Child with Respiratory Dysfunction

Ch. 27, 28

Chapter 27

Respiration problems till age 9 are #1 reason for admission to hospital.

Objectives:
 Anatomical Differences – same structures, but not as well developed till age 12 so more problems with respiration
 Signs and Symptoms of resp distress – treat the patient in distress before they reach respiratory failure.
 Therapies – similar to adults but different doses
o Considerations – not always cooperative, methods to ensure same effects
 Pediatric Resp. Acquired conditions
 Pediatric Resp. Chronic conditions

A&P Review of the Respiratory Tract:

 Upper Airway – sinus, nose, tongue, pharynx, larynx, and upper part of trachea
o The UA diameter is less subject to constriction than LA – relatively rigid due to cartilage.
 Lower Airway – trachea, bronchi, bronchiole, and alveoli (below cricoid cartilage)
o Contain little cartilaginous support, LA is mostly made of smooth muscle which is better able to constrict and dilate
the airway.
 We can use medications to change the structure to optimize the airway.
o Bronchiolar smooth muscle is highly reactive – can cause life-threatening obstruction during bronchospasm
 The clinical manifestations for each airway are similar regardless of which structure is the cause
o All structures are subject to obstruction from edema or foreign objects, but the degree of obstruction from
constriction of smooth muscle differs.

Pediatric Differences:
 The child’s respiratory anatomical differences affect the body’s response to disturbances in respiration
o A small amount of mucus in a small airway will obstruct the airway worse than an older child.
o If the child is surfactant deficient, they have decreased compliance.
 Upper Airway in Infant/Child:
o Smaller nares and nasopharynx – can easily be occluded during infection
 Increased airway resistance
o Smaller oral cavity with larger tongue
 Increased risk of obstruction
o Glottis and epiglottis are floppier and longer
 More prone to edema
o Larynx and glottis are higher in the neck
 Increasing the risk of aspiration
o Lymph tissues (tonsils and adenoids) grow rapidly in early childhood (hypertrophied) and will atrophy after the age
of 12
o Because fewer muscles are functional in the upper airway, it is less able to compensate for edema, spasm, and
trauma
 The large amounts of soft tissue and loosely anchored mucous membranes lining the airways increase the
risk of edema and obstruction
o Effects of 1 mm of circumferential edema:
 Small neonate – larynx in 4 mm diameter and 2 mm radius; if 1 mm of edema developed, it will halve the
airway radius and increase resistance by a factor of 16
 Older child – larynx is approximately 10 mm in diameter and 5 mm radius; the 1 mm of edema will reduce
the radius by 20% and increase the resistance by a factor of 2.4
 Narrow airways can cause greater increase in airway resistance
  Lower Airway in Infant/Child:
o Children’s airway diameter is smaller in size
o The infant’s airway cartilage is soft and compressible
 May cause the trach to more easily collapse during neck extension
o The trach division of the right and left bronchi is higher with a more acute angle
 There is a difference in the angle of access to the trachea at various ages
 Nurse must consider when the infant or child is positioned for resuscitation and airway clearance
o More pliant rib cage due to high compliance
 Rib cage can be easily distorted
o Infants and young children do not have as many alveoli as adults
 Increasing the risk for obstruction and atelectasis
o Infants are belly breathers, meaning they rely on diaphragmatic-abdominal breathing

Changes and Grows:

 The alveoli increase with age, so as they get older, they might grow out of the respiratory disease.
 Till age 12, mouth cavity gets bigger and tongue is smaller – less easy to obstruct.

Alterations are Structural and/or Functional:

 Thoracic Cavity – even diseases that aren’t respiratory related can affect the thoracic cavity (ex. Scoliosis)
 Lobes of the Lungs
o Lung volume varies according to posture – upright increases volume
o Increased compliance makes lungs abnormally easy to inflate with loss of elastic recoil (asthma)
o Decreased compliance means lungs are abnormally stiff or have difficulty inflating (respiratory distress syndrome)
o Infiltration of the alveoli can either prevent inflation (surface-active surfactant) or prevent gas exchange (fluids)
 Pleura
o Pleural space may contain:
 Air – pneumothorax
 Fluid – pleural effusion
 Serum – hydrothorax
 Blood – hemothorax
 Pus – pyothorax, empyema
o Inflammation of the pleura causes painful friction of pleurisy during respiratory movements
 Chest
 o Lung trauma
o Chronic over-inflation can cause a change in the anteroposterior diameter
 Ex: asthma and CF can produce barrel chest
o The volume of air inhaled increases with the growth of lungs, and the amount of CO2 increases
 Airways
o Dilation and constriction
o Change in airway resistance - increases in respiratory disease

Assessment of Respiratory Function:

 Position of comfort - lung volume varies depending on posture
o Upright position – lung volume is increased due to evenly distributed weight of abdominal contents causing
uniform application of negative intrathoracic pressure; elevates rib cage
o Supine position – lung volume is decreased because abdominal contents apply weight to create non-uniform
distribution of positive pressure to the diaphragm to the rib cage; pulls in the rib cage
 Vital signs – nurse needs to know the ranges for age groups
o RR, HR, BP, Temp
 Lung Auscultation
o Rales/crackles – result from the passage of air through fluid or moisture
o Wheezes – produced as air passes through narrowed passageways, regardless of cause
o Rhonchi – low-pitched, rattling lung sounds resembling snoring; due to obstruction or secretions in the larger
airway
 Respiratory Effort – are any of these present?
o Retractions – a sinking in of soft tissues relative to the cartilaginous and bony thorax
 Suprasternal – indicates severe airway obstruction
 Subcostal – indicates a flattened diaphragm due to increased intrathoracic pressure
 Intercostal – normal in infants, intercostal muscles act as stabilizing force

o Nasal flaring – a sign of respiratory distress and is a significant finding in infants

 Describe as minimal or marked
o Head bobbing in infant – sign of dyspnea
 Infant’s effort to ventilate by repositioning themselves
o Stridor – high-pitched, noisy respiration; usually indicates narrowing of the upper airway by obstruction or
secretion
o Grunting – the body’s attempt at more efficient respirations (UA)
 Characteristic of respiratory distress in newborns and infants
 A sign of pain in older children – suggesting acute pneumonia or pleural involvement
o Wheezing – continuous musical sound originating from vibrations in narrowed airways; primarily heard on
expiration (LA)
 Infants may have wheezing due to increased airway resistance and compliant chest wall
 If in older children, indicates lower respiratory tract infection as a result of inflammation,
bronchospasm, and accumulated secretions
o Clubbing – proliferation of tissue about the terminal phalanges
 Caused by extensive chronic hypoxemia due to cardiac defects or chronic pulmonary disease (cystic
fibrosis)
 The greater the angle, the more pronounced the clubbing
 Color Changes
o Pallor, mottled, and cyanosis are all important color changes
 Acrocyanosis – peripheral bluish discoloration resulting from circulatory stasis in newborn; unimportant
 Mottling and pallor are significant and usually indicate cardiopulmonary disease
  Cough – note if voluntary or involuntary; productive or non-productive?
o Auscultate for their breath sounds – sound is there then its gone after they cough
 Behavior Changes
o Changes in LOC
o Lethargic?
 Family History
o Frequent infections
o Asthma or cystic fibrosis
 Cardinal Signs of Respiratory Failure/Obstruction:
o Increased pulse and drop in RR
o Nasal flaring
o Retractions
o Increased restlessness

Nursing Management:
 Obtain history – onset, s/s, family history of respiratory illness
 Monitor cardio-respiratory status – pulse ox, EKG monitor
 Provide emotional support
 Discharge planning and home care teaching

Nursing Interventions:
 Ease respiratory effort – mechanical movement or medications (dilate bronchi)
 Fever management – body’s natural mechanism to fight infection
o Don’t automatically treat because it may just be a response to stress or dehydration. Too quick to treat the infection
– stopping the body’s natural way to decrease infection
 Promote rest and comfort
o “Cluster care” – manage care so not going in room every five minutes
 Infection control
o Hand washing
o Implement contact or droplet precautions – instruct the parents to follow the rules
 Promote hydration and nutrition
o Clear the airway before a feeding.
 Family support and teaching
 Prevent spread of infection – education
 Provide support and plan for home care

Diagnostics and Labs:

 Diagnostic Procedures:
o Bronchoscopy – visualizes the structures; invasive; involves general anesthesia
o Chest radiography (CXR) – can provide lots of information
 Can see atria and ventricles as well as vasculature
 Can see atelectasis in lungs
 Can see any fractures or penetration of bony structures
 We can look at lung fields and the airway
o Polysomnography – sleep study
 Obesity in pediatric population – look for sleep apnea
o Pulse oximetry – measures the percentage concentration of oxygen in blood continuously
 Make sure that the reading is correct.
o Spirometry – pulmonary function test
 Measures function of the lung, chest wall mechanics, and lung capacity to rule out any lung problems
o Sweat Chloride Test – gold standard for diagnosing CF
 Laboratory Tests:
o Arterial Blood Gas analysis – measures level of O2, CO2, and acidity in the blood
 High O2; High CO2 – deals with low respiratory rate
o Cultures – obtain aspiration and assess on a petri dish
o Neonatal screening for cystic fibrosis – blood test to look for IRT in the blood
 IRT is normally found in small numbers, but it is raised in CF.
o Protein-purified derivative (PPD) – tests if person has been exposed to tuberculin proteins
 Mantoux Test – intradermal injection of tuberculin to test immunity

Respiratory Therapy
  Oxygen Therapy – indicated for hypoxemia
o Oxygen is a drug and should be administered as prescribed
o Various oxygen delivery devices are used to give supplemental oxygen to children, and are selected to match the
concentration of oxygen needed and child tolerance of the device
 They are generally humidified in order to moisten airways – this loosens secretions and prevents drying
and injury of respiratory structures!

Oxygen Administration:
 Cannula – indicated for older infants/children who require lower concentrations; usually well tolerated but needs to be
secured
o Advantages:
 Provides low-flow and high-flow oxygen
 High-flow = 5-6 L/min
 Patient can be held by caregiver and be fed or receive routine cares (make sure to secure the child as they
may be prone to movement)
 Child able to eat and talk while receiving oxygen
 Able to observe patient more completely because nose and mouth aren’t obstructed
o Disadvantages:
 Must have patent nasal passages, may cause irritation
 May caused abdominal distention/discomfort or vomiting
 Difficulty controlling oxygen concentrations if child breathes through mouth
 Inability to provide mist if desired
 Masks – indicated for children; often not well-tolerated; must be correct size for patient’s face to create a good seal
o Simple – fits over patient’s nose and oxygen is delivered as the child breathes; for short-term oxygen therapy; flow
rates of 5-10 L/min
 Risk of carbon dioxide rebreathing
o Nonrebreather – has two one-way valves allowing higher oxygen concentrations to be delivered; generally used for
short time; flow rates > 4 L/min
 Used for acute hypoxia
o Not well-tolerated by children because the fit must be snug to the face to ensure adequate oxygen delivery
o Advantages:
 Various sizes available – comes in pediatric sizes
 Delivers higher oxygen concentration than cannula
o Disadvantages:
 Skin irritation, accumulated moisture on face
 Possibility child has fear of mask or suffocation
 Possibility of aspiration of vomitus
 Difficulty controlling precise oxygen delivery concentration
 Plastic Hood – indicated for premature/neonates/infants and is well-tolerated
o It is a reservoir that surrounds the head or body and provides a controlled oxygen-enriched environment for
respiration
 Between 21-100% of oxygen can be delivered
 At least 7 L/min of flow is necessary to maintain oxygen concentrations and remove exhaled CO2
 Need to have compliant lungs, want them no lower than 92%
o Advantages:
 Achievement of high oxygen concentrations (FiO2 <1.00)
 Free access to patient’s chest for assessment
 Can carry out most procedures without interruption of oxygen delivery
 Used more often to provide high humidity; less commonly used to provide oxygen to infants
o Disadvantages:
 Not tolerated by children who are old enough to push it off
 High humidity environment
o Don’t blow cold air into the face of an infant wearing a hood – can cause bradycardia
 Tent (rarely used today) – used in the past for older infants and some small children; surrounds the perimeter of the bed,
isolating the child
o The concentration of oxygen is difficult to maintain above 30-50%
o Must keep tent closed to maintain oxygen concentration
o Advantages:
 Achievement of lower oxygen concentrations (FiO2 <0.3-0.5)
 Child able to receive increased inspired oxygen concentrations even while eating
o Disadvantages:
 Necessity for right fit around bed or crib to prevent leakage of gas
  Cool and wet tent environment due to humidification – watch their skin
 Poor access to patient; inspired oxygen levels fall when tent is entered
 Child may not tolerate it around the bed/crib
 They are restricted to movement – sensorimotor is delayed.
o Management:
 Minimize openings
 Leave toys with the child
 Keep warm by monitoring temperature
 Tent linens can become wet, so check frequently and make sure patient is dry
 Use judgement when removing the child from the tent – depends on the situation

Aerosol Therapy – effective in depositing medication directly into the airway

 Bronchodilators, steroids, mucolytics, and antibiotics, suspended in particulate form, can be inhaled so that the medication
reaches the small airways
o Can be aerosolized or nebulized with air or with oxygen-enriched gas
 Hand-held nebulizers – common; medicated mist discharged from a reservoir into a small plastic mask held over the child’s
nose; older children may use mouthpiece instead of mask
o Instruct child to take slow, deep breaths through an open mouth during treatment
 Metered Dose Inhalers (MDI) – self-contained, hand-held device that allows for intermittent delivery of a specified amount
of medication; used by children with asthma or CF
o A spacer device or holding chamber attached to the MDI can help to coordinate breathing and aerosol delivery.
o Spacer should be attached to an MDI when an inhaled corticosteroid is administered – to prevent yeast infections in
the mouth (if child is too young to rinse mouth post-treatment)
o Spacer Considerations:
 Take 4-6 normal breaths
 Hold breath for 5-10 seconds
 Slowly exhale through pursed lips
 Rinse everyday with warm water
 Dry

Bronchial (Postural) Drainage – indicated whenever excessive fluid or mucus in the bronchi is not removed by normal ciliary
activity and cough
 Postural Drainage – positioning the child to help move mucus and facilitate removal of secretions
o Performed 3-4x /day after other therapy
o Done before meals, wait at least one hour after meals
o Several position options
o Lasts 20-30 minutes
o Pediatric considerations – should be done before they are fed because they are at increased risk for aspiration
 Chest Physiotherapy (CPT) – the use of postural drainage in combination with airway clearance techniques (vibration,
percussion, cough)
o Breaks up all the mucus to help get it out of airways
o Manual percussion - cupped hand only over rib cages (do not slap)
 Can also use a soft circular mask – adapted to maintain air trapping
o Should be performed 30 minutes to 1 hour after bronchodilator is administered
o Direct older children to deep breath and exhale through the mouth rapidly and as completely as possible; inspirations
after the activity often stimulates a deep, productive cough
o Incentive Spirometry can also be employed to stimulate deep breathing
 For young children, it is difficult to get them to take deep breaths
 May incorporate play such as having them blow bubbles instead
Artificial Airways – commonly used in association with mechanical ventilation and in children with upper airway obstruction
 Oropharyngeal Airway
 Nasopharyngeal Airway
 Endotracheal Tube – intubated nasally, orally, or through direct tracheal routes
o Uncuffed ET tubes are typically used in children < 8 years of age
o Although infants have been successfully maintained on ET tubes for longer periods, tracheostomy is considered in
infants/children who require intubation for extended period
o Not uncommon to attempt early weaning to prevent tracheomalacia
o Switching to trach:
 Allows child to speak and eat; also facilitates clearing of secretions
 Most severe complication related to immediate intubation is hypoxia with accompanying bradycardia
o Closely monitor patients during intubation attempts and if hypoxia occurs, discontinue procedure until vital signs are
stable
o Reinstitute BVM and oxygen

Mechanical Ventilation – necessary if child’s respiratory status is deteriorating and the respiratory effort is excessive or inadequate
 Used to adjust alveolar ventilation (PaCO2 and pH), to improve oxygenation (PaO2 and pulse ox), and to decrease work
of breathing (patient assessment)
 Temporary assistance: bag-valve-mask (BVM) – self-inflating bag with a mask and non-returnable valve to prevent
rebreathing
 Noninvasive Positive Pressure Ventilation – for pediatric patients who have respiratory difficulties in acute, chronic, and
home settings
 Invasive (positive pressure) mechanical ventilation requires an artificial airway – either a ET tube or a tracheostomy
 Indications for consideration:
o Inadequate ventilation caused by apnea, CNS injury/ infection, alveolar hypoventilation, respiratory muscle
weakness, medication toxicity, foreign body obstruction
o Excessive work of breathing manifested by retractions, tachypnea, decreasing oxygen saturation, abnormal
respiratory patterns
o Inadequate respiratory effort
o Hyperventilation for treatment of increased ICP

Tracheostomy – permanent airway for long-term health in infants and children

 Pediatric tubes are constructed with more of an acute angle and they soften at body temperature to conform to the contours of
the trachea
o Materials resist formation of crusted respiratory secretions, thus made without an inner cannula
 Safe Nursing Standards:
o At bedside, have a trach the same size and a smaller size, trach ties, and obturator (used to slide trach in)
o Make sure the trach is secure
o Provide fresh trach care and suctioning
 Risk factors:
o Dislodging trach
o Obstructing airway
o Bleeding
o Suctioning may cause hypoxia – make sure to give patient time to recover between suctions
 Tracheostomy Care:
o Fresh trach care: sutures remain at least 5 days
 Ensure that the ties are tight enough to not cause decannulation but not too tight to cause
o Monitor for post-op complications:
 Hemorrhage, decannulation, edema, and obstruction
o Assess stoma area:
 Observe for signs of infection and breakdown of skin
 Keep skin clean and dry
 Gently remove secretions around stoma with warm soap and water or saline
o Administer supplemental oxygen with a humidification system to prevent drying of the respiratory mucosa
 Or be put on a ventilator for respiratory support
o Suctioning: vacuum pressure set to 60-100 mmHg for infants and children; even lower pressure for premature
 Insert catheter 0.5 cm beyond or just at the end of the trach tube
 Don’t put in until you meet resistance, could cause traumatic lesions to the trachea
 Don’t suction more than 5 seconds in infants, 10 seconds in children. Let recover for 30-60 seconds so
saturation can return to normal
 Aseptic technique in hospital, clean technique at home
 o Change trach ties (one finger rule): have another nurse to assist. Change at least once a day or whenever they are
soiled
o Accidental decannulation requires immediate tube replacement
 If a child has malformed or flexible tracheal cartilage, this may cause the airway to collapse when the tube
is removed or dislodged
 If airway is too tight, insert a smaller tube
 Provide BVM to stoma – if ineffective, the stoma can be occluded with a gloved finger while another HCP
does BVM ventilation to the mouth/nose
o Can incorporate developmental support in care – child swings and walkers, taking child out for routine outings
o Prior to discharge, parent must demonstrate competency in trach care
 Suctioning the trach
 Cleaning the stoma
 Changing the collar, ties, or trach tube
 Adapting to home environment
 Using durable medical equipment
 Recognizing warning signs of obstruction, infection, or a worsening condition
 Things needed at the bedside: suction, ambu bag, one trach the infant’s size, +1 size smaller, obturator

Ch. 28

Respiratory Infections
 Etiology and Characteristics
o Accounts for the majority of acute illnesses in kids
o There is a lower rate in infants younger than 3 months – passive immunity from the protective function of maternal
antibodies
 Nursing Care of the Child with a Respiratory Tract Infection
o Ease Respiratory Efforts:
 Warm or cool mist is a common therapeutic measure for symptomatic relief of respiratory discomfort -
soothes inflamed membranes
 Mist tents are used in the hospital to humidify air and relieve discomfort
 Steam from running a shower may help but is not evidence-based
o Promote Rest and Comfort:
 Limit activity – encourage febrile children to rest and engage in quiet activities
o Clear airway with a bulb syringe, especially before feeds
o Possible need for Neosynephrine drops but not more than ordered (about 3 days) to prevent rebound congestion
o Prevent Spread of Infection:
 Careful hand-washing
 Cover cough/sneeze with a tissue or hand
 Dispose of tissues properly and wash hands
 Do not share drinking cups, washcloths, or towels
 Keep infected children out of contact with other children, school, or day care settings to prevent spread
 Teach children to stay away from ill children, wash their hands frequently, and avoid eating or drinking
from same utensils or cups
o Reduce Temperature:
 Assess if parent’s know how to take child’s temperature and help them administer antipyretic
 Some require more instruction than others
 Emphasize accuracy of calculating doses
 Give ibuprofen with food or milk to avoid stomach upset
 Encourage cool liquids to reduce temperature and minimize chances of dehydration
o Promote Hydration:
 Infants are especially prone to fluid/electrolyte deficits during a respiratory illness because a rapid RR and
presence of fever precludes adequate fluid intake
 Parents can encourage adequate fluid intake by offering frequent small amounts of favorite fluids (clear
liquids if vomiting)
 IV fluids may be required for short term to reestablish hydration
 Advise parents to observe frequency of voiding and notify nurse if there is insufficiency
 Counting number of wet diapers in a 24-hour period is a satisfactory method of assessing output in
nonhospital settings and if not acutely ill
 Urine output should be 1 ml/kg/hr in a child < 30 kg; 30 ml/hr if child > 30 kg
o Provide Nutrition:
  Children can be permitted to determine their own need for food
 Urging food in anorexic children may precipitate N/V and cause an aversion to feeding
 In acute period of illness, maintaining hydration with encouragement of fluids is of greater importance
o Provide Family and Support Care
 Teaching about home medications – antipyretics, nose drops, antibiotic therapy
 Emphasize importance of giving the full course of the antibiotic, regardless if the child appears ill
 Teach parent what to avoid:
 Avoid steam vaporizers – hazardous, potential for burns
o Instead run hot shower for 10-15 minutes for steam effect
 No OTC cough/cold medications for kids younger than 4
 Avoid Vicks Vaporub – can increase inflammation and secretions (can cause local irritation)
 Don’t give honey to infant less than 1 – risk for botulism

Upper Respiratory Tract Infections (URIs)

 Nasopharyngitis – the “common cold”
 Caused by numerous viruses – RSV, rhinovirus, adenovirus, influenza, and parainfluenza
 Clinical Manifestations:
o Fever – varies with age of child
o Irritability, restlessness
o Decreased appetite and fluid intake
o Nasal inflammation
o Vomiting and diarrhea
 Home Management – varies with age
 Sounds Associated with URIs:
o Stridor – coarse, seal-like (hospitalized immediately if stridor at rest)
o Grunting – expiratory sounds

Croup Syndromes:
 Involves swelling or obstruction in the area of the larynx characterized by barking or brassy cough and hoarseness
 Small children and infants are at greater risk due to smaller airway diameter
o Primarily children 6 months to 3 years from late autumn to early winter
 Characterized by hoarseness, a “barking” cough, inspiratory stridor, and varying degrees of respiratory distress
o Affects the larynx, trachea, and bronchi
 Described by anatomic area that is primarily affected:
o Epiglottitis (or supraglottitis), laryngitis, laryngotracheobronchitis (LTB), and tracheitis
 Acute Epiglottitis – medical emergency; serious obstructive, inflammatory process affecting the epiglottis; not seen as often
due to immunizations
o Causative agent: H. influenza
o Usually occurring between ages 2-5 years
o Potential for complete respiratory obstruction (supraglottic) – VERY SERIOUS EMERGENCY
o Clinical Manifestations:
 Sudden, abrupt onset
 May have had prior sore throat or cold symptoms
 Dysphagia
 Look sicker than they sound
 Inspiratory stridor – frog-like croaking on inspiration
 Mild hypoxia, distress, fever
 Cherry red swollen epiglottis, red inflamed throat
 Thumb Sign – enlarged, rounded epiglottis and narrow airway
 Pale, cyanosis
 Tripod positioning to keep glottis open (chin thrust out, mouth open, tongue protruding)
 Patient is positioned upright and leaning forward
 Drooling, agitation, absence of spontaneous cough ****
 Don’t want to swallow – protect from aspiration
 Appears frightened
 Possible retractions – suprasternal or substernal
 May progress to decreased LOC, acidosis, and sudden death
o Therapeutic Management:
 Potential for complete respiratory obstruction
 Prevention – Hib vaccine (influenza type B vaccine)
o Nursing Alerts:
 Tripod position
  Epiglottitis image – thumb sign; enlarged, rounded epiglottis with a narrow airway

Nursing Considerations/Management:
 Treat as emergency if suspected, be prepared to tube (nasotracheal or tracheotomy is possible)
 Position for comfort – let patient sit in parent’s lap
o Don’t agitate them so they don’t cry.
 Decrease anxiety
 Do NOT examine with tongue blade unless prepared to intubate
o Can precipitate further obstruction
 NO IV unless you are prepared to intubate or trach
 Keep suction at the bedside
 Keep emergency respiratory equipment at bedside
 Experienced personnel must accompany patient to radiology for lateral neck x-ray
o Let child stay in parent’s lap during x-ray if needed
 Trach if patient is in severe respiratory distress (or having nasotracheal intubation)
 Administer humidified oxygen
 Maintain close monitoring
 Administer antibiotic therapy if suspected bacterial epiglottitis
o Give as IV first and continue as oral
o Possible need for corticosteroids
o Edema usually goes down after 24 hours of antibiotics
 Prevention: influenza type B vaccine

Acute Laryngotracheobronchitis – viral invasion of upper airways

 Causing agents: RSV, influenza A and B, parainfluenza, M. pneumoniae
o Starts as a URI then spreads, causing the mucosal lining of the trachea and larynx to become inflamed  airway
becomes narrowed making it hard to inhale air past the obstruction
 Incident:
o Most common type of croup experienced by children admitted for hospitalization
o Usually found in kids younger than 5
 Signs and Symptoms:
o “Had a cold originally and woke up then next day with a barky cough”
o Gradual onset of low-grade fever
o Gets worse at night and when crying
o Inspiratory stridor when the airway becomes very narrow, along with retractions
o Cough
o Hoarseness
o Nasal flaring
o Tachypnea
o Cyanosis
o Severe obstruction can cause respiratory acidosis and respiratory failure
 Care/Management Goal: maintain airway and adequate respiratory exchange
o Home if mild:
 Teach parents the signs and symptoms of respiratory distress and when to seek help
o Cool mist (high humidity) leads to constriction of edematous blood vessels
 Cool air vaporizer, cool night air
 Not enough substantial evidence that it really works
 Encourage beverages they like
 Hospital Care:
o Mist tent
o Make effort to consume drinks they like
o IVF if poor oral intake
 NPO if tachypneic RR >60 (20-30 minute feeds)
o Hold infants and small children upright when feeding
o Allow parents to stay with child  decreases anxiety  decreases respirations
o Monitor blood gases – if illness is progressing and need concrete data
o Continuous pulse ox and cardo-respiratory monitoring
o Frequent assessment – vigilant observation and accurate assessment of respiratory status (MOST
IMPORTANT NURSING FUNCTION)
o Ensure that emergency equipment at the bedside
 o Observe for signs and symptoms of impending airway obstruction: increased RR, increased HR, retractions,
restlessness, nasal flaring
o Allow child time to rest to their conserve energy
o Provide reassurance for the parents
 Home Management – always the goal
 Pharmacologic Treatments for URI
o OTC
o Antihistamines
o Antipyretics
o Cough suppressants
o Decongestant
 Acute LTB Medication Administration:
o Racemic epinephrine (Vaponephrine) – alpha-adrenergic effects cause mucosal vasoconstriction which decreases
subglottic edema
 Rapid onset but may take a couple of hours to see max effect
 May need to be repeated every 20-30 minutes as needed
 Most commonly used for laryngoedema
o Corticosteroids – anti-inflammatory; oral route is effective
o Budenoside nebulizer with IM Decadron – if child is unable to tolerate PO dose
o Heliox – mixture of helium and oxygen that may reduce the work of breathing and relieve airway obstruction;
respirable gas, lower density, less airway resistance; though not proven to be more effective
 For severe LTB

Tonsillitis – inflammation of the tonsils

 Tonsils are very vascular – filter and protect respiratory and alimentary tracts from invasion of pathogenic organisms
 Palantine tonsils (located on either side of the oropharynx) are removed during tonsillectomy
 Pharyngeal or adenoids located on posterior wall of the nasopharynx are sometimes removed
 Causative agent: either viral or bacterial
o Group A β-hemolytic streptococci (GABHS)
 Diagnostic evaluation – rapid antigen testing and culture
 Etiology and Risks in Children
o Tonsillitis occurs with pharyngitis
o Because of the abundant lymphoid tissue and the frequency of URIs, tonsillitis is a common cause of illness in
young children
 Risk for serious sequelae
o Acute rheumatic fever
o Acute glomerulonephritis
o Scarlet fever (though rarely seen in United States)
 Clinical Manifestations:
o Inflammation causes symptoms
o Tonsils become swollen (2+ common in kids) and can obstruct passage food or air
o Enlarged adenoids can make breathing hard because they block the nares and the child starts mouth breathing
o Mouth breathing dries mucus membranes
o May have cough, dysphagia, mouth odor, tonsils red and inflamed, fever, and snoring
 Therapeutic Management
o Surgery is controversial. There is risk involved. Not done if:
 Cleft palate
 Acute infection
 Systemic diseases, especially bleeding disorders
 Avoided in kids less than 3 years because of excessive blood loss in young kids and possibility of regrowth
o Tonsillectomy done if:
 Recurrent frequent strep infections
 Peritonsillar abscess or recurrent pharyngitis
 They are so large that it interferes with airway/eating
 Malignancy
o Adenoidectomy done when nasal breathing is obstructed
o Tonsillectomy
 Surgical considerations
 Postop
 Important discharge teaching
 Nursing Care Management:
 o Treat the symptoms
o Comfort care:
 Soft to liquid diet
 Cool mist vaporizer to keep mucus membranes moist
 Saltwater gargles, lozenges
 Tylenol, may need something stronger
 Antibiotics if bacterial (though cause is usually viral)
 Surgical intervention – controversial
o Strep:
 Give penicillin – oral or IM (Penicillin G)
 Erythromycin if patient has penicillin allergy, or other antibiotics

Tonsillectomy:
 Pre-Op – assess for possibility of bleeding tendencies (clotting times) and infection (URI) – controversial
 Postop
o Airway: positioning – lie on abdomen or side until fully awake
o Bleeding:
 Observation – frequent swallowing?
 Prevention of recurrent bleeding (do not drink out of straws)
 Maintain quiet environment
 Minimize agitation and crying
 NO suctioning
 Avoid coughing, clearing throat, or blowing nose
 Inform parents that dried blood, some blood-tinged mucus, or old clots are normal
 Inspect secretions/emesis for blood
 Dark brown, old blood may be in emesis
o Comfort:
 Ice collar for pain
 Analgesics (avoid oral), give at regular intervals
o Diet:
 NPO until awake and no sign of hemorrhage
 No red or brown fluids/meds, no straw
 Cool water, diluted juice (not citrus)
 Avoid milk products (the coating makes kids want to clear throat), then advance 
 Soft food (jello, soup)
 Very important: assess for frequent swallowing and notify surgeon immediately
o Oxygen and suction at bedside
o Post-op hemorrhage (can occur up to 10 days after surgery)
o Frequent swallowing increased HR, pallor, frequent throat clearing, restlessness, late sign is decreased BP
o Surgery may be required to cauterize or ligate a bleeding vessel

Family Teaching:
 Postoperative hemorrhage – can occur up to 10 days after surgery; s/s of bleeding: frequent swallowing, increased HR, pallor,
frequent throat clearing, decreased BP (late sign)
 Avoid irritating foods – seasoned, citrus, crackers
 Avoid vigorous tooth brushing
 Avoid gargles
 Avoid coughing, clearing throat, putting sharp object in mouth
 Analgesics or ice collar for pain
 Limit activity for 1-2 weeks

Acute Streptococcal Pharyngitis – strep throat

 Etiology and Risks in Children:
o Group A beta-hemolytic streptococci (GABHS)
o Children are at risk for serious sequelae – acute rheumatic fever, acute glomerulonephritis, and scarlet fever (rarely
seen in US)
o Expected in children over the age of 2 who have pharyngitis even if no exudate is present
 Clinical Manifestations:
o Ranges from asymptomatic to severe toxicity
o Abrupt onset
o Characterized by pharyngitis, headache, fever, and abdominal pain
o Tonsils and pharynx may be inflamed and covered in exudate (50-80%) – appears 2nd day of illness
 o Anterior cervical lymphadenopathy (30-50%) occurs early; nodes often tender
o Pain relatively mild to severe (enough to make swallowing difficult)
o Clinical manifestations usually subside in 3-5 days, unless complication occurs
 Diagnostic Evaluation
o Rapid antigen testing – obtained by vigorous swabbing of both tonsils and posterior pharynx
o Throat culture – usually used for confirmation if rapid test kit comes back negative but the s/s of infection are still
present
o One or both are done to rule out GABHS
 Therapeutic Management:
o If strep sore throat infection present - oral Penicillin prescribed to control acute local manifestations and maintain
adequate level for 10 days (eliminates organisms responsible for later s/s)
 Does not prevent the development of GABHS, but prevents the spread
o Amoxicillin – given QD for 10 days is just as effective as Penicillin
o IM penicillin G Benzathine is also appropriate therapy, but it is painful
 Some preparations contain penicillin G procaine as well to reduce pain
o Oral erythromycin indicated for children allergic to penicillin
o Other drugs that treat GABHS pharyngitis:
 Erythromycin, clarithromycin, azithromycin, clindamycin, oral cephalosporin, and amoxicillin with
clavulanic acid
 Nursing Care Management

Lower Respiratory Infections

 Sounds Associated with LRIs – heard through auscultation:
o Wheezes
o Rails/crackles
o Rhonchi
o Pleural rub

Bronchiolitis – viral infection affecting bronchioles; usually occurring in winter and spring
 RSV (Respiratory Syncytial Virus) – responsible for large majority of bronchiolitis
 Incidence:
o Most frequent cause of hospitalization by one year
o Most kids have had RSV by 3 years
o Peak incidence is between 2-7 months of age
o Usually begins in the fall, peaks in winter, decreases in spring
o The younger the infant, the greater the likelihood that it will be severe enough to warrant hospitalization
 Pathophysiology:
o Affects epithelial cells of respiratory tract
o Swelling of bronchial mucosa, increased mucus and exudate clogs bronchioles
o May have air trapping
 Transmission:
o Direct contact with respiratory secretions – hands, nose, mouth, eyes
o Can also be spread by large particle aerosols but no documentation of airborne transmission
o Transmitted thru direct contact with contaminated secretions and potentially through inhalation of droplets
 Generated from cough or sneeze
o Can survive for hours on hard surfaces, gloves, or tissues
o Survives 30 minutes on skin
 Initial Clinical Manifestations:
o Starts with URI symptoms (runny nose, low grade fever)
o Cough
o Wheezing
o Possible osteomyelitis or conjunctivitis
 Clinical Manifestations with Progression of Illness:
o More coughing, more wheezing, retractions, crackles, dyspnea
o Tachypnea - RR increased >70 breaths
o Fever
o Decreased oral intake
o Copious secretions
 Clinical Manifestations of Severe Illness:
o Tachypnea >70 breaths/min
o Listlessness
o Apneic spells
 o Decreased breath sounds and poor air exchange
o Cyanosis
 Can lead to pneumonia  decreased oxygen levels and increased CO2 levels  respiratory failure, apnea, and pulmonary
edema
 Diagnosis:
o Obtain nasal or nasopharyngeal secretions
o ELISA (enzyme linked immunosorbent assay) – determine definitive diagnosis
 Therapeutic Management:
o Maintain airway
o Bronchodilator – beta-2 agonist
o Humidified oxygen
o Hydration

Nursing Management of Bronchiolitis:

 Initial Management
o Generally, treat symptoms; usually managed at home
o Administer cool humidified oxygen
o Possible need for bronchodilator
o Ensure adequate fluids (PO or IV)
 RSV Prevention for High Risk Patients – Medication Administration
o Ribavirin – the only specific therapy approved for hospitalized children; controversial for infants due to high cost,
aerosol route, potential toxic effects among HCPs, and conflicting results of health care trials
 Administered by aerosol via hood for 12-20 hours
 Teratogenic
o Prophylactics for high-risk patients: the only product available for prevention of RSV Synagis (Palivizumab) –
given once monthly between November and march
 Usually IM but can be IV
 Candidates: high risk individuals
 Premature, infants/children with chronic lung disease, infants/children with CHD,
immunocompromised
 Contact precautions
 Appropriate assignments – assign nurses working with RSV patients to not work with non-RSV patients (prevent spread)
 Place patient on isolation to reduce excessive person contact with the patient; could possibly room together with another RSV
patient
 Hand washing!
 Provide bulb suction with normal saline drops for infants with RSV – copious nasal secretions which makes breathing and
bottle feeding difficult
o Parents should learn how to instill normal saline drops into the nares and suction the mucus with bulb syringe before
feedings and bedtime
 Hydration/nutrition – may offer small amounts frequently (5-10 cc each time)
 Monitor pulse ox

Foreign Body Aspiration:

 Risk among young children – most common in ages 1-3
o Major offenders – hot dogs, round candy, peanut, grapes
 Varying degrees of obstruction
 Pathophysiology:
o Most inhaled foreign bodies lodge in a main-stem or lobar bronchus, few find the distal portions of the lung field,
and the remaining lodge in the trachea.

 Clinical Manifestations:
o Dyspnea, cyanosis
o Inability to speak
o Cough, stridor, hoarseness
 o Wheezing, stridor
o Some may not have symptoms depending on degree of obstruction; others may have recurrent problems
o Manifestations will correlate with where it is lodged
 Major Offenders – grapes, peanuts, hotdogs
 Diagnostic Evaluation:
o History
o X-ray
o Bronchoscopy
 Medical Management:
o Abdominal thrusts, back blows (CPR)
o Removal by basic life support
 Nursing Management:
o Recognize signs and symptoms
o Back blows and abdominal thrusts
o PREVENTION TEACHING – essential anticipatory guidance
 Small children shouldn’t be allowed access to small objects
 Hazards of aspiration in relation to developmental level of the child
 Encourage parents to teach children safety
 Caution parents about behaviors child may imitate – ex: holding foreign objects like pins, nails, or
toothpicks near their lips or mouth)

Asthma – chronic inflammatory disorder of the airways; episodic bronchial hyper-responsiveness

 Limited airflow or obstruction that reverses spontaneously or with treatment
 Etiology and Pathophysiology:
o Most common chronic disease of childhood but can be controlled
o Severity varies from intermittent to severe and persistent
o Incidence is increasing
o Three major groups:
 Early childhood
 Chronic, related to allergies
 Related to obesity and early puberty in girls
 Pathophysiology:
o Asthma results from complex interactions among inflammatory cells, mediators, and cells and tissues present in
airways
o Inflammation contributes to increased airway reactivity
o Three major things happening:
 Mucus membranes inflamed and edematous
 Secretions from mucus glands accumulate
 Bronchospasms
o Narrowed airway due to bronchospasms, mucosal edema, and mucus plugging results in resistant airway  air
becomes trapped
o More pronounced difficulty during expiration than inspiration
o Exacerbations are episodes of progressively worsening SOB, cough, wheezing, chest tightness
 Hypoxemia can occur due to miss matched ventilation and perfusion

Asthma Severity Classification in Children 5 and Older:

 Stage I – mild, intermittent asthma
o Symptoms less than 2 days per week
o Nighttime symptoms (awakenings): none for ages 0-4 and < 2x per month for ages 5-11
 Stage II – mild, persistent asthma
o Symptoms more than 2x per week, but less than once per day
o Interference with normal activity: minor limitation
o Use of short acting B-agonist for symptoms control: more than 2 days a week but not daily
 Stage III – moderate, persistent asthma
o Daily symptoms
o Interference with normal activity: some limitation
o Use of short acting B-agonist for symptom control: daily
 Stage IV – severe, persistent asthma
o Continual symptoms throughout the day
o Interference with normal activity: extremely limited
o Use of short acting B-agonist for symptom control: several times a day
Manifestations are often preceded by a URI:
 Asthma can be aggravated by allergens, exercise, infections, fragrances, stress, etc.

Clinical Manifestations of Asthma:

 Prolonged expiration
 Classic – dyspnea, wheezing, cough (particularly at night)
 May start with itching to the neck/upper back
 SOB
 Restless, irritable, or tired
 May hear rhonchi
 May progress to:
o Audible wheeze with prolong expiration
o Color changes
o Anxious
o Sweating
o Tight chest
o Accessory muscles
o Too much effort to speak
o Does not want to lie down
o Decreased breath sounds

Asthma Continued:
 Diagnostic Evaluation: based on clinical manifestation
o Pulmonary Function Tests - to evaluate severity of overall disease
 Peak inspiratory flow meter – max flow of air exhaled in one second (compare to personal best)
 How is the patient at that moment?
 Monitors patient’s breathing capacity
 Therapeutic Management – goal is to maintain normal pulmonary function/prevent chronic symptoms and exacerbations
o General – regular doctor visits, avoid triggers, maintain hydration
o Allergen control and avoidance
 Controlling cockroaches - exterminating live cockroaches, carefully cleaning kitchen floors and cabinets,
putting food away after eating, and taking trash out in evening
 Sensitized persons should be careful about getting pets – cat and dog dander relate to asthma development
 Pollutants (smoke, pesticides, mold, nitrogen dioxide, sulfur dioxide, etc.) believed to contribute to asthma
morbidity in children – avoid and minimize
 Exposure to tobacco smoke has significant contributing factor to asthma in infants and small children; also
a trigger of episodes
o Drug Therapy
o Promote self care
o Support child and family

Drug Therapy to Prevent/Relieve Bronchospasm:

 Long-term control medications:
o Inhaled corticosteroids – anti-inflammatory effects
 First line therapy in children over 5
 Inhalation used long-term
 Take after bronchodilator if ordered
o Cromolyn sodium – NSAID, blocks early and late reactions
 Nebulizer
o Xolair – monoclonal antibody; for older children, admin subcutaneous once or twice a month in children 12 or older
 Limits degree of release of allergen modifiers
o Leukotriene modifiers – blocks inflammation and bronchospasm effects by mediating inflammatory response that
leads to airway responsivity
 Montelukast = singulair
 Accolade (Zafirlukast) take in evening
o Long-Acting B2-Agonists – allows smooth muscle to relax
 Serevent BID is a preventer – NOT TO BE USED AS RESCUE MED
 Used for children 12 and older
o Methylxanthines (Theophylline) – not used as much, can be toxic
 Monitor serum levels
o Nedocromil
 Quick-relief (rescue) medications:
 o Short-Acting B2-Agonists – MDI or nebulizer
 Not to be used 3-4 x per day
 Concerns regarding increased use; known MOA and S/E
 Proventil (Albuterol), Levabuterol (Xopenex), Terbutaline
o Anticholinergics – blocks bronchoconstriction
 Ipratropium
o Systemic corticosteroids are used as quick relief (or rescue) medications
o Magnesium sulfate – IV smooth muscle relaxer that decreases inflammation
 Watch respirations
 Monitor for hypotension (emergency)
 Drug Routes:
o Inhaled for most medications
 MDI with spacer (significance?)
 Nebulizer
o Oral and IV - less effective in treating asthma

Maintain Health and Prevent Complications:

 Breathing exercises and physical therapy
 CPT
 Hypo-sensitization – allergy shots (epi pen)
 Exercise Induced Bronchospasms (EIB) – rare if child engages in short bursts of energy
o Appropriate exercise = short bursts of energy; prolonged may cause exercise induced asthma
o Swimming is excellent
 Asthma Medicine Plan pg. 1279:
o Green/good 80-100%
o Yellow/caution 50-80%
o Red/danger < 50%

Status Asthmaticus
 Respiratory distress continues despite vigorous therapeutic measures (bronchodilators); medical emergency
o Concurrent infection in some cases
 Clinical Manifestations:
o Profuse sweating
o Sits upright
o Sudden agitation or quietness
o Can’t rest
 Therapeutic Intervention:
o Emergency treatment: Epinephrine 0.01 ml/kg subq (max dose 0.3 ml)
o IV magnesium sulfate
o IV ketamine
o IV corticosteroids
o Heliox (most aggressive)
 Nursing Care:
o PICU
o Administer humidified oxygen
o Cardio-respiratory monitoring
o Monitor Pulse ox
o Respiratory assessments
o Administer meds:
 Terbutaline subq, magnesium sulfate
 Heliox – for difficulty with ventilation (usually see effects within 20 minutes)
 Bronchodilators every 20-30 minutes x3 or continuous
o Monitor effectiveness of meds
o Maintenance IVF for hydration
o Positioning
o Emotional support
o Assist with tests
o Teaching for outpatient management:
 Avoid allergens
 Recognize signs and impending attack
 PEFM
 Make sure use MDI correctly
  Spacers
 Use and side effects of B2 adrenergic
 Avoid respiratory infections
 Drug free asthma treatment using CPAP
 Reactive Airway Disease
o Sometimes used as another term for asthmas, but not necessarily the same time
o May be used to describe history of wheezing, coughing, SOB with no known cause
o Not a specific diagnosis but often given to younger kids because it’s hard to diagnose kids less than 6 years with
asthma

Cystic Fibrosis (CF):

 Exocrine gland disorder that causes glands to produce thick, tenacious mucus which can obstruct major organs
(lungs, pancreas, small intestine, liver)
o They can’t handle their sodium, so all of their mucosal areas are very thick and occlude regularly.
 Autosomal recessive genetic disease – abnormal gene located on the long arm of chromosome 7
o 95% known cases occur in Caucasian
o Most common lethal genetic illness among Caucasian children
o Both parents must be carriers
o ¼ chance of occurring
o Approximately 3% of US whites are symptom-free carriers
 Clinical Manifestations vary widely and change as the disease progresses

 Pathophysiology:
o Infectious Pathogens:
o Pseudomonas aeruginosa, Burkholderia cepacia, Staphylococcus aureus, Haemophlus influenza, Escheria coli,
Klebsiella pneumoniae
o Can alter the function of organs – multisystem involvement
o Excessive excretion of sodium and chloride from sweat and salivary glands
o Thick mucus production especially in lung
o Can affect fertility
o Respiratory tract and pancreas are predominately affected
o Respiratory problems:
o Mucus obstruction of airways – mechanical
o Thick, inspissated mucuprotein accumulates, dilates, precipitates, and coagulates to form concretions in the
glands and ducts
o Excellent medium for bacterial colonization especially P. aeruginosa (hard organism for CF kids to fight), S.
auresus, B. cepacia, and H. influenza
o Chronic infection leads to progressive fibrosis
o Pulmonary hypertension
o Pneumothorax – increased RR and HR, dyspnea, pallor, cyanosis, decreased bowel sounds
o GI problems:
o Pancreas secretes thick mucus that obstructs pancreatic ducts
o Interferes with secretion of pancreatic enzymes/prevents them from reaching duodenum
o Leads to impaired digestion and absorption of nutrients (fats, proteins, even carbs to a lesser extent)
o Impairs absorption of ADEK
o Poor absorption leads to steatorrhea
 o Poor secretions of salt and water can cause an intestinal obstruction
o Thick secretions block ducts  cystic dilation  degeneration  diffuse fibrosis
o Prevents pancreatic enzymes from reaching duodenum
o Impaired digestion/absorption of fat  steatorrhea
o Impaired digestion/absorption of protein  azotorrhea
o Endocrine function of the pancreas initially stays unchanged
o Eventually pancreatic fibrosis occurs; may result in diabetes mellitus
o Focal biliary obstruction results in multilobular biliary cirrhosis
o Impaired salivation
o Endocrine problems:
o Pancreatic fibrosis can cause a decrease in the number of islets of Langerhans
o 13% have CF related diabetes
o Reproductive problems:
o Men often sterile
o Women may have problems conceiving due to mucus blocking cervical canal
o Sweat gland problems:
o Excessive salt loss from intense heat or exercise

Presentation:
 Wheezing respiration; dry, nonproductive cough
 Generalized obstructive emphysema
 Patchy atelectasis
 Cyanosis
 Clubbing of fingers and toes
 Repeated bronchitis and pneumonia
 Meconium ileus
 Distal intestinal obstruction syndrome
 Excretion of undigested food in stool – increased bulk, frothy, and foul
 Tissue wasting
 Prolapse of the rectum
 Delayed puberty in females
 Sterility in males
 Parents report children taste salty
 Dehydration
 Hyponatremic/hypochloremic alkalosis
 Hypoalbuminemia

Clinical Manifestations:
 Vary widely and change as the disease progresses
 Pancreatic enzyme deficiency
 Chronic progressive obstructive lung disease
 Sweat gland dysfunction
 Respiratory:
o Wheezing
o Chronic cough
o Thick sputum
o Dyspnea
o Barrel-shaped chest
o Clubbing
o Frequent respiratory infections
o Hemoptysis: life threatening if over 250 ml/24 hrs
o Pneumothorax
o These are present in almost all patients with CF, but onset/extent is variable
o Stagnation in mucus and bacterial colonization result in destruction of lung tissue
o Tenacious secretions are difficult to expectorate – obstruct bronchi/bronchioles
o Gradual progression follows a chronic infection
 Bronchial epithelium is destroyed
 Infection spreads to peribronchial tissues, weakening bronchial walls
 Peribronchial fibrosis
 Decreased oxygen/ carbon dioxide exchange
o GI:
  Meconium ileus usually first sign in 15-20% of newborns with disease
 Impaction/obstruction
 Distal obstruction syndrome (bowel obstruction causing pain, N/V)
 Bulky stool
 Steatorrhea
 Frothy, foul stools that float
 Weight loss due to poor absorption even if good appetite
 Reflux
 Rectal prolapse
 Pancreatic enzyme deficiency
 Progressive COPD associated with infection
 Sweat gland dysfunction
 Failure to thrive
 Increased weight loss despite increased appetite
 Gradual respiratory deterioration
o Reproductive – delayed puberty
o Integumentary – taste salty

Diagnostic Evaluation:
 Early infancy screening
 DNA identification of mutant genes – presence of CFTR gene
 H&P exam – early infancy or childhood
o Meconium ileus – cannot pass meconium
o FTT
o Chronic respiratory infections, absence of pancreatic enzymes
o Increased electrolyte concentration
o Chronic pulmonary involvement
 Sweat chloride test – stimulating sweat production with a special device and collecting the sweat on filter paper to
measure the electrolytes
o Collected twice
o Normal < 40
o Suspicious 50-60
o CF if >60
 CXR – patchy atelectasis and obstructive emphysema
 Stool analysis – requires 72-hour sample with accurate recording of food intake

Medical Management Goals:

 Prevent/minimize pulmonary complications
 Ensure adequate nutrition for growth
 Encourage physical activity
 Promote reasonable quality of life

Respiratory Management:
 Postural therapy/CPT – cornerstone of pulmonary therapy
o Remove excessive mucus secretions
 Bronchodilators administered before CPT
 Forced expirations
 DNase/Pulmozyme – decreased viscosity of mucus
 Nebulized hypertonic saline can increase mucus clearance
 Regular daily exercise
 Treat pulmonary infections immediately and aggressively – some like pseudomonas may not be able to eradicate but can
control
o Antibiotics – may be given prophylactically inhaled but once organisms are established, IV is better
 Need a higher dose because it metabolizes the antibiotics more rapidly
o PIC/CVL for IV antibiotics can allow IV to be delivered at home
 Assess/prevent pneumothorax
 Annual flu vaccine
 Montelukast
 Daily ibuprofen – NSAID
o Slows rate of pulmonary function decline
 Monitor for hemoptysis
  Lung transplantation is used as the final option

GI Management:
 Pancreatic enzymes:
o Replace pancreatic enzymes
o Make sure food is mixed with digestive enzymes in duodenum
o Give with meals and snacks, usually 1-5 tablets within 30 minutes before eating
o If enteric coated – do not crush, prevents neutralization by gastric acid
 Capsules (whole or sprinkled)
o Adjust to achieve normal growth and development and to decrease stools to 1-2/day
 High protein, high calorie carb diet, unrestricted fat
o 110%-200% standards for healthy people
o Three meals and three snacks per day
o Increase calories when sick
o Water miscible ADEK
o Fat and salt are needed in diet – salt supplementation
o Possible enteral or parenteral feedings
o Prevention/early management of intestinal obstruction:
 Administer stool softener
 Lactulose – may abort early distal intestinal obstruction
o Watch for rectal prolapse – want to see reduction
o Oral glucose – lowering agents or insulin injections as needed
o Diet and exercise management
o Ensure adequate fluids

Family Support:
 Coping with emotional needs of child and family
 Child requires treatments multiple times per day
 Frequent hospitalization
 Implications of genetic transmission of disease
 Home care and support

Prognosis of CF:
 Decreased life expectancy for child born with CF
 Maximize health potential
 Nutrition
 Prevention/early aggressive treatment of infection
 Pulmonary hygiene
 New research—hope for the future
o Transplantation
o Gene therapy
o Bilateral lung transplants
o Improved pharmacologic agents

Nursing Care:
 Pulmonary assessment
 GI assessment
 Assist with diagnostic tests
 Provide emotional support
o Coping with emotional needs of the child and family
o Child requires treatments multiple times per day with frequent hospitalizations
o Implications of genetic transmission of disease
o Home care and support
 Good hand washing
 Covering receptacle for soiled tissues
 Aerosol therapy/CPT/oxygen
 Increase calories when sick
 Skin care especially after toileting
 IVF
 Assist with meal planning
  Teach CPT and postural drainage
 Breathing exercises and games
 Coordinate for home antibiotics
 Immunizations up to date
 Anticipatory guidance
 Goals:
o Prevent/minimize pulmonary complications
o Adequate nutrition for growth
o Assist to adapting to chronic illness
 Prognosis:
o Decreased life expectancy for child born with CF
o Maximize health potential:
 Nutrition
 Prevention and early aggressive treatment of infection
 Pulmonary hygiene
o New research – hope for the future
 Transplantation
 Gene therapy
 Bilateral lung transplants
 Improved pharmacologic agents

Respiratory Distress Syndrome (RDS) – leading cause of respiratory failure in premature

 Serious lung disorder caused by immature thorax and inability to produce surfactant
 Hyaline membrane formed (necrotic cells from alveoli and fibrin)
o Decreases elastic quality of lung
o Stiffer lungs require more pressure to expand

Bronchopulmonary Dysplasia (Chronic Lung Disease) – persistence of lung disease following premature birth and respiratory
support provided in the neonatal period
 Surfactant deficient
 Usually develops in neonates treated with oxygen and positive pressure ventilation for respiratory failure or RDS
 Pathological Process seen in premature and LBW babies:
o History of mechanical ventilation, oxygen dependency, and abnormal pulmonary radiographic findings at 2
days of life
o May be secondary to RDS in which the child required high concentration oxygen
o Some initial injury (prenatal infection, mechanical ventilation, oxygenation) to already immature lung leads to
chronic inflammation process that results in recurrent injury and abnormal healing
o High oxygen concentration interferes with ciliary activity making it hard to clear lungs of mucus, this
aggravates airway obstruction
o Decreased surface area for oxygen
o Hard to wean from ventilator
 Diagnostic Evaluation:
o Radiographic findings – hyper-expansion and atelectasis
o Oxygen therapy or PPT after 28 days
o Signs of respiratory distress (tachypnea, irritability, flaring, retractions, grunting, FTT, wheezing, crackles,
pulmonary edema, cyanosis)
o History of requiring mechanical ventilation in the first week of life for more than 3 days
 Medical Management:
o Prevention of at risk infants
 Good prenatal care
 Give mom medications before delivery
o Take caution with vent settings / HFOV
o Possible trach
o Lowest level oxygen with gradual weaning from vent
o Fluid control and restriction
 Very susceptible to fluid overload and pulmonary edema
 Diuretics – control interstitial fluids
o Palivisumb (Synagis) to prevent RSV
 Nursing Care:
o Allow for rest – cluster your care
o Small frequent feedings – strict feeding schedule high in calories
 Possible fluid restriction – at risk for pulmonary edema
o
 Monitor for over-hydration and under-hydration
o Involve parents in care
 Home oxygen therapy once gaining weight and oxygen needs to be lowered
 May go home with trach and on a ventilator
 Tracheostomy and ventilator training
 Teach parents CPR
 Prognosis: developmental delays, growth failure, risk for respiratory infections, risk for lower IQ, special education,
speech therapy, physical therapy

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