Pediatric Disorders

Childhood Disorders

MLNG CELESTE,RN, MD

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Respiratory Disorders

Anatomy and physiology of respiratory tract • Origin of respiratory system: > arises as an out pouching from the pharyngeal pouch; this gradually migrates to the thoracic area ( completed by the 3rd fetal month) > right primary bronchus is slightly larger than the left and form obtuse angle (FB) > larynx is 1/8 the adult size at birth > trachea is 4cm long in newborn, 1/2 of adult’s

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> at birth the bifurcation is located at the 3rd or 4th thoracic vertebra > at 4 years, it is at the level of T5 > at 12 years, it is located between T5 to T6 > ethmoidal and maxillary sinuses are present at birth > Frontal and sphenoidal sinuses do not develop until 6-8 years of age

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>after 2 years of age the R bronchus is shorter and more vertical than the left > change in thoracic breathing begins at 2-3 years of age and is complete at 7 > in infants, the wall of the airway has less cartilage (advantageous in an infant – an infant does not develop bronchospasm- less smooth muscle in the airway), therefore wheezing cannot be a prominent finding

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Adult vs Pediatric Airway • • • • •

Smaller airway diameter 4mm Distance between structures is shorter Organisms move faster, usually viral Infants obligate nose breathers “Little finger”=trachea size

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• Respiratory system is divided into 2 divisions: 1. upper respiratory tract a. nose b. Para nasal sinuses c. pharynx d. larynx e. epiglottis 2. lower respiratory tract a. bronchi b. bronchioles c. alveoli

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MLNG CELESTE,RN, MD

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• Inspiration : - delivers warm and moistened air to the alveoli transports O2 across the alveolar membrane to hgb –laden RBC allows CO2 to diffuse from RBC back to alveoli thru expiration , CO2 filled air is discharged to outside

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Respiratory center > medulla of the brain receptors: 1. peripheral receptor-aortic arch and carotid arteries( stimulated by decrease PO2) 2. central respiratory receptor- stimulated by: a. increased PCO2 b. decreased temp. c. body acidity d. increased BP

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Assessment of respiratory illness in children • Physical assessment: A. cough- a reflex initiated by stimulation of nerve of respiratory tract mucosa by the presence of dust chemicals, mucus or inflammation - useful procedure to clear excess mucus paroxysmal cough- refers to series of expiratory cough after deep expiration ( seen in whooping cough, aspiration ) MLNG CELESTE,RN, MD

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B. rate and depth of respiration: tachypnea > increased RR, 1st indicator of airway obstruction in young children C. Retraction> inward drawing of the chest causes: airway obstruction, stiff lungs non compliant lung > commonly seen in infant and newborn because intercostal tissue is weaker and less developed MLNG CELESTE,RN, MD

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• Supraclavicular or Suprasternal retraction Suggests upper airway obstruction • intercostal retractions suggest lower airway obstruction

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D. Restlessness : - when infantS or children have decreased O2, they become anxious and restless E. Cyanosis – blue tinge to the skin - becomes apparent when PO2 is < 40mmhg resulting to increased RR

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F. Clubbing of fingers: - change in the angle between the fingernail and nailbed because of increased capillary growth in the fingertips G. Adventitious sounds: - extra or abnormal breathing sounds - I/E ratio is reversed (bronchial or tubular breathing)

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MLNG CELESTE,RN, MD

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types of adventitious sounds: 1.rhonchi- obstruction at the level of nose or pharynx 2.stridor- obstruction at the level of larynx (best heard in supine position) 3. wheezing – obstruction is in the lower trachea or bronchioles (expiration) 4.rales –when alveoli are filled with fluids, fine crackling sounds

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H. Chest diameter: - in children with COPD unable to exhale completely air trapped in lung - alveoli (hyperinflation) , produces elongated A-P diameter of the chest ( pigeon chest)

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• Laboratory tests: A. blood gas analysis > an invasive method for determining the effectiveness of ventilation acid-base status > provides information about oxygenation of the blood as well as O2 saturation of Hgb

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causes of decreased O2 saturation 1. pt with respiratory distress (O2 cannot reach the bloodstream) 2.Hgb is defective, cannot carry a full complement of O2 PCO2 - measures efficiency of ventilation PCO2 - seen in children who are hyperventilating (breathing deeply) blowing off to much PCO2 PCO2- seen in children who are hypoventilating (breathing very shallow) can’t blow off the CO2 MLNG CELESTE,RN, MD

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CO2 retention( due to obstruction/ or hypoventilation) partial pressure of CO2 rises and concentration of CO2 in the plasma increases respiratory acidosis ( the body compensates by increased kidney tubular reabsorption of HCO3 (bicarbonate) respiratory alkalosis ( bicarbonate exceeds the amount of acid in the blood)

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in doing ABG- we need arterial blood (reflects how the lungs are oxygenating the blood) if venous blood : only reflects oxygenation of particular extremity from which blood is drawn SITES commonly used for ABG: 1. temporal artery- young infant 2.umbilical artery- newborn 3.radial artery- older children Other sites: (small infant) if impossible to obtain arterial blood directly, heel or finger stick may be used (just warm the area for about 20 minutes) MLNG CELESTE,RN, MD

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B. Pulse oximetry - continuous ,non invasive technique for measuring O2 saturation How to use: 1. sensor and photo detectors are placed around vascular bed 2. infrared light is directed through the finger from the sensor to the photo detector( hgb absorbs light wave differently when bound to O2 )

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Advantages: 1. non-invasive 2.continuous monitoring by pulse oximeter allows you to modify the care Disadvantages: 1. sensor is small ( needs frequent checking) 2. excess light may distort the reading

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ABG values: PH7.35-7.45 < 7.35 - acidosis, > 7.45 - alkalosis PCO2- 35-45 mmHg < 35 alveolar hyperventilation and respiratory alkalosis > 45 means ventilatory failure and respiratory acidosis PO2 –

80-100 mm Hg 60 -80 mmHg- mild hypoxemia 40-60 mmHg - moderate hypoxemia < 40 mmHg - severe MLNG CELESTE,RN, MD

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explanation: > ph, >

ph,

pco2 - alkalosis pco2 - acidosis

how to know if metabolic or respiratory? >references are ph and HCO3  if ph and HCO3 - both elevated it is metabolic  If ph and HCO3 is - opposite direction (one is increased, the other is decreased) it is respiratory ( SMOR)

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C. Nasopharyngeal culture D. Respiratory syncytial virus nasal washing > pt is placed in supine position and 1-2 ml of sterile saline is dropped with sterile needleless syringe into one nose, then nose is aspirated using small, sterile bulb syringe E. Sputum analysis: >rare in children younger than school age

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• Diagnostic tests: 1. chest X-ray 2. bronchography 3.pulmonary function test - measures the forces of inertia, elasticity, and flow resistance measured by the use of spirometrydevice that records the force of air exchange

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Therapeutic techniques Expectorant therapy 1.Coughing 2.Chest physiotherapy - 3 technique are involved 1. postural drainage 2.percussion 3. vibration 3.Mucus clearing devices- by use of flutter device- stainless steel ball inside the device moves when the child breathes out -causing vibration in the lung -helps loosen the mucus MLNG CELESTE,RN, MD

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Therapy to improve oxygenation • O2 administration: via hood, nasal cannula, and O2 mask • Pharmacologic therapy: 1.antihistamine 2.decongestant-causes vasoconstriction leading to shrinkage of mucous membrane - expands breathing space

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MLNG CELESTE,RN, MD

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3. expectorant- helps raise mucus 4. bronchodilator- used to open lower airway 5. metered dose inhaler- route of medication • Incentive spirometry - device that encourages children to inhale deeply to aerate the lung • Breathing technique- blowing ball, blowing out with purse lips • Tracheostomy -opening in the trachea to create an artificial airway to relieve respiratory obstruction • Endotracheal tube intubation

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MLNG CELESTE,RN, MD

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Disorders of upper respiratory tract • • • • • •

Choanal atresia Acute nasopharyingitis Pharyngitis Tonsillitis Epistaxis Sinusitis

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• • • • • •

Laryngitis Congenital laryngomalacia/tracheomalacia Croup Epiglottitis Aspiration Bronchial obstruction

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Choanal atresia > congenital obstruction of the posterior nares by an obstructing membrane or bony growth, preventing newborn from drawing air through the nose into the nasopharynx > either unilateral/bilateral Dx. Can be assessed by holding the newborn’s mouth closed, then gently compressing 1st nostril, then the other

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• Sign: cyanosis • Treatment:1. local piercing of the obstructing membrane 2. surgical removal of the bony growth

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Acute nasopharyngitis (common colds-coryza) • • • •

Most frequent infectious disease in children Average of 10-12 colds/ year Incubation period- 2-3 days Causes: 1. rhinovirus-most common cause 2.coxsackie virus-most common cause 3.respiratory syncitial virus (major etiologic agent) 4.adenovirus 5. parainfluenza /influenza virus

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• Signs and symptoms: 1.nasal congestion 2.watery rhinitis 3.low grade fever 4. mucus membrane is edematous 5.cervical lymph nodes may be swollen and palpable 6. body malaise

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• Pathophysiology: > initial pathology is submucosal edema of nasal mucosa by shedding of ciliated epithelial cells (5th day) nasal mucopurulent discharge > interferon ( plays major role in recovery)

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• Treatment: Common colds is self-limiting > supportive care > relief of nasal obstruction- use of isotonic saline drops and aspiration > antipyretic or analgesic agents > antitussive is sometime used for persistent cough

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• Nursing diagnosis: parental health-seeking behaviors related to management of child’s cold symptoms

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Pharyngitis > it is an inflammation of mucous membrane of the throat and involves the nasopharynx, uvula and soft palate > peak incidence occurs between 4 to 7 years of age > either bacteria or viral, occurs as a result of a chronic allergy

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• Viral pharyngitis: > causative agent is virus signs and symptoms: 1. sore throat 2. fever 3.general malaise Physical examination: 1. enlarged lymph nodes 2. erythema on the back of the pharynx

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• Treatment: 1. antipyretic 2. gargle with warm water- school age 3. provide liquid foods- (+) difficulty of swallowing Nursing diagnosis: > risk for fluid volume deficit

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Streptococcal pharyngitis: > caused by group A beta-hemolytic streptococcus > can lead to cardiac and kidney damage > more severe than viral infection Signs and symptoms: 1. marked erythema of the back of throat and palatine tonsils 2. tonsils are enlarged and (+) white exudates in the tonsillar crypts 3. high grade fever 4. difficulty swallowing MLNG CELESTE,RN, MD

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- Spread by infected nose or throat mucus through coughing or sneezing - Dx: throat swab and culture

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treatment: 1. antibiotics- 10 days course of oral antibiotics( pen G or clindamycin) 2. high fluid intakes 3.relief of pain Complications: 1. Rheumatic fever 2. Glomerulonephritis

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Retropharyngeal abscess > limited to young infants > infected lymph nodes that drain the nasopharynx located behind the posterior pharyngeal wall Signs and symptoms: 1. sore throat 2. fever 3. enlargement of regional lymph nodes

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laboratory findings: leukocytosis- increased WBC count treatments: 1. antibiotics( benzathine pen G. penicillin V 2. abscesses resolve on their own if not - can do incision 3.adequate fluid intakes

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Tonsillitis > term commonly used to refer to infection and inflammation of palatine tonsils palatine tonsils- located on both sides of pharynx Adenitis- refers to infection and inflammation of the adenoids ( pharyngeal) tonsils Adenoids- located in the nasopharynx Tubal tonsils- located at entrance to the eustachian tube Lingual tonsils-located at base of tongue MLNG CELESTE,RN, MD

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Signs and symptoms: 1. difficulty of swallowing 2.fever 3.lethargy 4.mouth breathing- (posterior pharyngeal obstruction) 5.difficulty hearing- (Eustachian tube obstruction) 6.halitosis 7. sleep apnea

MLNG CELESTE,RN, MD

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Causes: > child <3 years old-often viral > school age children- group A betahemolytic Treatment: 1. antipyretic 2. analgesics 3. antibiotics 4. if recurrent onset of tonsillitis can do tonsillectomy

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Tonsillectomy-removal of palatine tonsils Adenoidectomy- removal of pharyngeal tonsils-can be done in children having sleep apnea Indication for tonsillectomy: > chronic tonsillitis (not done if inflamedbecause may spread infection) Nursing diagnosis: pain related to surgical procedure

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Epistaxis (nosebleed) > extremely common in children and usually occurs in trauma > occurs during respiratory illness, after strenuous activity Management: 1. keep patient in upright position with head tilted slightly forward to minimize the amount of blood pressure in nasal vessels , keep blood moving forward not back to nasopharynx

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2. apply pressure to the side of the nose with your fingers 3. keep the child quiet and stop crying because crying increases pressure in the blood vessels of the head and prolonged bleeding 4. control of bleeding, can give epinephrine ( 1:1000) to constrict blood vessels 5. can put nasal packing for continued pressure MLNG CELESTE,RN, MD

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Sinusitis > rare in children younger than 6 years of age because frontal sinuses do not develop fully until 6 years of age > occurs as secondary infection in older children Causes: streptococcal staphylococcal H- Influenza

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signs and symptoms: 1. fever 2. purulent nasal discharge 3. headache 4.tenderness over the affected sinus

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treatment: 1. antipyretic 2.analgesic 3. antibiotic for specific infection 4. nasal spray- Oxymetazoline HCl (afrin) shrinks the edematous mucus membrane and allows infected material to drain 5. warm compress Complication if untreated: otitis media and osteomyelitis ( involves facial bone)

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Laryngitis >inflammation of larynx > occurs as complication of pharyngitis or from excessive use of voice Management : 1.sips of fluid offer relief from annoying tickling sensation 2. rest the voice for at least 24 hrs

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Congenital laryngomalacia/tracheomalacia >means laryngeal structure is weaker than normal and collapses more than usual in inspiration > produces laryngeal stridor present from birth, intensified when infant is in supine or sucking Sign and symptoms: 1. stridor 2.retraction- on inspiration because of increased effort needed to pull the air into trachea from collapse cartilage ring

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management: no routine treatment -instruct parents to feed the child slowly

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Croup ( laryngotracheobronchitis) > inflammation of the larynx, trachea, and major bronchi > one of the most frightening diseases of early childhood for both parents and children > common cause organism is viral infection usually parainfluenza virus

MLNG CELESTE,RN, MD

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signs and symptoms: 1. low grade fever 2. barking cough at night 3. inspiratory stridor 4.retractions Management: > provide warm, moist environment- as an emergency measure > give corticosteroids (nebulizer)reduces inflammation and bronchodilation > intravenous hydration Nursing dx.: Ineffective airway clearance related to edema MLNG CELESTE,RN, MD

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Epiglottitis > inflammation of the epiglottis( flap of tissue that covers the opening to larynx to keep out food and fluids > its an emergency because the swollen epiglottis can’t rise and allow the airway to open

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causes: 1. H-Influenza type b 2. pneumococci, streptococci - most common cause 3. echovirus 4.respiratory syncitial virus Signs and symptoms: 1. begins as mild URTI 2. 1 to 2 days (+) severe inspiratory stridor 3. high fever 4. hoarseness 5. sore throat 6. dysphagia MLNG CELESTE,RN, MD

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never illicit gag reflex- may cause complete obstruction Laboratory findings: 1. leukocytosis ( 20,000-30,000 mm3) with the proportion of neutrophils Management: 1. provide warm moist environment 2. O2 administration 3. antibiotic therapy 4. intravenous therapy MLNG CELESTE,RN, MD

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Aspiration > inhalation of foreign objects into the airway > common in infant and toddlers Management: Heimlich subdiaphragmatic abdominal thrust ( stand behind the child and place a fist just under the child diaphragm)

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*if the child is lying on his back at the time of aspiration, stand at the head of the table or bed, place hand under child diaphragm and exert inward and upward thrust* * for infant, use back thrust to dislodge an aspirated object-turn the infant prone over your arm and administer up to 5 quick back blows forcefully bet. The infants shoulder blades using the heel of the hands*

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Bronchial obstruction > obstruction on the bronchus (commonly on the right) > common in child younger than school-age Sign and symptoms: 1.violent cough 2.hemoptysis 3.fever 4. purulent sputum 5.localized wheezing 6.dyspnea MLNG CELESTE,RN, MD

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Laboratory findings: 1.radioopaque finding on chest X-ray 2.leukocytosis Complications: 1. pneumothorax 2.atelectasis 3. lipid pneumonia

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management: 1. bronchoscopy –removal of foreign body 2. close observation after bronchoscopy, frequent V/S monitoring 3. maintain on NPO after procedure, assess for gag reflex

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Disorders of lower respiratory tract

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Influenza > inflammation and infection of the major airway > caused by orthomyxoviruses, influenza type A, B, C Sign and symptoms: 1.cough 2. fever 3.fatique 4.achings pain 5.sore throat 6. GIT symptoms-diarrhea, vomiting MLNG CELESTE,RN, MD

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incubation period: 1-5 days Management: 1. antipyretic 2. antiviral drugs( tamiflu) 3. influenza vaccine( yearly given) Complication:1. bronchitis 2. pneumonia

MLNG CELESTE,RN, MD

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Bronchitis > inflammation of major bronchi and trachea > affect preschool and school age Sign and symptoms: 1. fever 2. cough 3.nasal congestion

MLNG CELESTE,RN, MD

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causes:1. influenza virus 2. adenovirus 3. mycoplasma pneumoniae Course of disease: 1-2 weeks Sign and symptoms: 1.fever 2. dry hacking cough 3. on auscultation can hear rhonchi and rales 4. On chest x-ray finding-diffuse alveolar ` hyperinflation and some marking on hilus of lungs

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Management:-1. aim in relieving the respiratory symptoms 2. antipyretic 3. adequate hydration 4. antibiotic therapy

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Bronchitis Etio

S/sx

Viral Rhinovirus,Influenza, RSV, Parainfluenza adenovirus, paramyxovirus

Rhinitis, cough, malaise, chills, mild fever, sore throat Dx Clinical Xray – normal Course Mild & self-limiting Rx supportive

Bacterial S. pneumoniae, S. aureus, H. Influenzae, M. Catarrhalis higher fever and a more prod cough Elev c-reactive CHON

Up to 2 weeks empirical; amoxMLNG CELESTE,RN, MD tetracycline,

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Bronchiolitis > inflammation of the fine bronchioles and small bronchi > common lower respiratory illness in children younger than 2 years of age , peak in 6 mos of age Causes: 1. adenovirus 2. parainfluenza 3. respiratory syncytial virus

MLNG CELESTE,RN, MD

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sign and symptoms: 1. nasal flaring 2.intercostal/subcostal retarction on inspiration 3. tachypnea 4. mild fever 5. expiratory wheezing 6. tachycardia 7. chest x-ray –pulmonary infiltrates or collapse of alveoli

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Management:1. antipyretic 2. adequate hydration 3. if in respiratory distress can hospitalized the pt. 4. O2 administration 5. nebulized with bronchodilators nursing diagnosis: > parental anxiety related to respiratory distress in child

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Respiratory syncytial virus > an RNA virus, common cause of bronchiolitis in young children. > can cause apnea/periodic halt of respiration > peak in severity between 48 to 72 hrs Sign and Symptoms: -lethargic, cyanosis, nasal flaring retraction, grunting, rales/ rhonchi Diagnosis: throat or nasal culture

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management: therapy is supportive 1. O2 administration 2.antiviral drugs( ribavirin) 3. isolate the patient 4. vaccination-RSV-IGIV and palivizumab

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asthma > an immediate hypersensitivity ( type 1) response, most common illness in children > condition may be intermittent, symptomfree period > common seen in children with atopy or tend to be hypersensitivity to allergens

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Mechanism of disease: > primarily affect the small airways and involve 3 processes: 1. bronchospasm-happen because of stimulation of PSNS (smooth muscle constriction) 2. inflammation bec. of mast cell 3. mucus production activation this will release histamine, leukotrienes & prostaglandin

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Signs and symptoms: 1. dry cough 2. expiratory wheezing 3. cyanosis 4. retractions

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Clinical Assessment of Asthma

MILD

MOD

SEVERE

talking Phrases usually agitated inc (+) audible

at rest words agitated

RR accessory wheeze

walking sentences may be agitated Inc (-) audible

pulse

<100

100-120

breathless talks in Alertness

MLNG CELESTE,RN, MD

>30 (+) audible w/o steth >120 96

Intermittent

Am sx

< 1x/wk

Mild Persistent >1x/wk

Pm sx


>/= 2x/mo

ModerateP ersistent Daily; aff activ >1x/wk

PEFR

80%


60-79%

<60%

PEFR var

< 20%

20-30%

30%

>30%

MLNG CELESTE,RN, MD

Severe Persistent Daily; dec activ > 1x/wk

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Ndx: • Ineffective breathing pattern r/t bronchospasm, edema and accumulation of mucus • Fear r/t sudden onset of Asthma attack • Activity intolerance r/t imbalance between O2 supply and demand • Health seeking behaviors r/t prevention and treatment for asthma attack

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Management • B2 agonists, Theophylline, Steroids, Cromolyn Na, O2 • Orthopneic position • Monitor VS, hydration • Adequate nutrition and non allergenic diet • Environmental modification • Health education

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Status Asthmaticus • Progression of attack unresponsive to therapy • Preceding upper respiratory tract infection, allergen, cigarette smoke • HR and RR inc, dec breath sounds, cyanosis Mx: cont B2 agonist and IV steroid, O2, coughing, fluids, monitor I and O, ET

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Pneumonia • Inflammation of lung parenchyma Bacterial and atypical pathogens: NB – GBS, Gr (-) bacilli, chlamydia Children – S. pneumoniae, H. Influenzae, M. catarrhalis Adolescents – S and M Pneumoniae, M. catarrhalis Hospital acquired – Pseudomonas, Klebsiella, E. Coli, Enterobacter sp Viral Pneumonia - RSV, Parainfluenza, Adenovirus, Influenza MLNG CELESTE,RN, MD

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Pathophx: aspiration/hematogenous/inhalation Alveoli inflammation hypoxemia S/sx: fever, chills, cough, irritability, poor feeding, restlessness, headache, GI sx, tachypnea, use of accessory muscles, decreased BS, crackles, dullness on percussion, chest retractions Dx: cxr, blood and sputum c/s MLNG CELESTE,RN, MD

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Parameter Bacterial

Viral

Atypical

Age

Any

Any

5-15 yo

Onset

Abrupt

Variable

Insidious

Fever

High

Variable

Low-grade

Toxicity

Toxic

Variable

Non-toxic

Inc RR

Common Common Uncommon MLNG CELESTE,RN, MD

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Perimeter

Bacterial

Viral

Atypical

Cough

Productive

Non prod

Non prod

Assoc sx

Mild coryza

Mild coryza

Bullous myringitis, pharyngitis

PE

(+) consolidation few crackles

variable

Fine crackles, wheezing

Inc WBC

common

variable

Uncommon

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• Nsg Dx: • Ineffective breathing pattern r/t physiologic effects of Pneumonia • - O2, monitor VS, CPT, encourage coughing and deep breathing • Risk for deficient fluid volume r/t diminished oral intake and increased insensible fluid losses sec to diaphoresis, dyspnea and fever • - weigh and monitor daily, sips of fluid, temp control, monitor I and O

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• Activity Intolerance r/t effects of Pneumonia and Tachypnea - rest periods, O2, small frequent feedings, gradual increase in activity, support and contact with family

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Tuberculosis • High incidence in infancy and adolescence • Primary Complex – based on xray; first encounter of a child w/ TB; Ghon’s tubercle w/ lymphangitis and regional (hilar) lymphadenopathy • Primary Pulmonary TB – clinical TB • d/t Mycobacterium Tuberculosis Dx: exposure, s/sx (2 or more), PPD, cxr MLNG CELESTE,RN, MD

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Classification of TB Class I TB exposure – (+) exposure Class II TB infection – (+) exposure and PPD Class III TB disease – active TB or 3 or more of criteria - Exposure, s/sx, (+) tuberculin test, xray, sputum exam, culture Class IV TB inactive – (+/-) hx of prev TB, (+/-) hx of prev hx of chemotherapy, (+) xray evidence of healed lesion and (+) PPD

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S/sx: fever, weight loss, cough, anorexia, night sweats, painless lymphadenopathy Tx: SCC Intensive INH, Rifampicin, PZA x 2 mos Maintenance INH, Rifampicin x 4 mos

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Respiratory Distress Syndrome • Preterm, infants of diabetic mothers, C/S, hx of asphyxia S/sx: inc RR, chest retractions, nasal flaring, expiratory grunt, cyanosis, HPN, hypothermia Dx: ABG, Xray Mgmt: surfactant replacement, O2, ventilation, supportive care MLNG CELESTE,RN, MD

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Decreased surfactant lungs collapse Inc pulmonary resistance blood shunts through F.O. and D.A. poor lung perfusion and gas exchange further decrease in surfactant

hypoxia acidosis

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Cardiovascular Disorders

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Fetal Circulation • placenta provides the exchange of gas and nutrient • four shunts in fetal circulation: – Placenta – Ductus venosus – Foramen Ovale – Ductus arteriosus

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Special Structures: Foramen Ovale Connects the left and right atrium Bypassing fetal lungs Obliterated after birth to become fossa ovalis Umbilical Vein Brings oxygenated blood coming from the placenta to the heart an liver Becomes ligamentum teres Umbilical arteries Carry unoxygenated blood from the fetus to placenta Become umbilical ligaments after birth Ductus venosus Carry oxygenated blood from umbilical vein to IVC Bypassing fetal liver Becomes ligamentum venosum after birth Ductus arteriosus Carry oxygenated blood from pulmonary artery to aorta Bypassing fetal lungs Becomes ligamentum arteriosum; closes after birth MLNG CELESTE,RN, MD

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Fetal Circulation

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Fetal circulation: oxygenated blood (placenta) umbilical vein liver

ductus venosus inferior vena cava right atrium

septum of blood)

foramen ovale (flap opening in the atrial that allow only R-L movement

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• Continuation: left atrium

(small amount)

left ventricle

aorta arteriosus supply the body

body

right ventricles

pulmonary arteries ductus aorta supply blood to the

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• Continuation: superior vena cava right atrium right ventricle pulmonary arteries ( ductus arteriosus) aorta supply blood to the body

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Common Congenital Heart Defects • abnormalities that develop before birth • can occur in the heart's chambers, valves or blood vessels • A baby may be born with only one defect or several that tend to occur in combination. • Of the dozens of heart defects, some are mild and may need minimal or no medical treatment even through adulthood, while others are lifethreatening, either immediately to the newborn or over time. MLNG CELESTE,RN, MD

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1.Acyanotic Heart Disease L R shunt high pressure to low pressure oxygenated to unoxygenated blood 2. Cyanotic Heart Disease R L shunt

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Classifications of CHD 1. Defects with increased pulmonary blood flow -ASD, VSD, PDA 2. Defects with decreased pulmonary blood flow - TOF, Tricuspid Atresia 3. Obstructive Defects - COA, Aortic stenosis, Pulmonic stenosis 4. Mixed Oxygenation/ defects -TOGV, Truncus Arteriosus, Hypoplastic left heart syndrome (HLHS) MLNG CELESTE,RN, MD

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ACYANOTIC HEART DISEASES

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Atrial septal defect • Similar to a ventricular septal defect, this is a hole that occurs when the septum separating the right and left atria doesn't close properly. • This allows blood from the left atrium to flow into the right atrium, instead of into the left ventricle and on to the aorta and the rest of the body. • The defect can cause several complications, including arrhythmias, heart failure, stroke and, in rare cases, pulmonary hypertension. • Minor cases may cause no symptoms and may not require treatment. • Larger defects may require surgical closure or cardiac catheterization MLNG CELESTE,RN, MD

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1. Atrial Septal Defect • • • •

Opening between atria females late childhood/early adulthood S/sx: cyanosis (CHF), dyspnea, fatigue, failure to thrive, split S2 • Mx: Antibiotics, surgery 1-3 yo • Cx: endocarditis, heart failure • Postop: monitor arrhythmia, administer antibiotics

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Ventricular septal defect

• Sometimes called a hole in the heart • most common congenital heart defect • occurs when the septum, the muscular wall separating the right and left ventricles, fails to fully form • The hole allows oxygen-rich blood to leak from the left ventricle into the right ventricle, instead of moving into the aorta and on to the body. • Too much blood may flood the lungs. • This defect can lead to heart failure, excessive blood pressure in the lungs (pulmonary hypertension), infections of the heart (endocarditis), irregular heartbeats (arrhythmias) and delayed growth. • Small holes may heal on their own or cause no symptoms. Larger holes may require surgical repair by stitching together or covering with a patch.

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2. Ventricular Septal Defect • Opening in ventricular septum • Most common • S/sx: respiratory infections, failure to thrive, dyspnea, fatigue, pansystolic murmur • Mx:closes spontaneously otherwise surgery <2 yo failure • Postop: monitor • Cx:pulmonary HPN, endocarditis, heart arrhythmia, administer antibiotics

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Patent ductus arteriosus • Before birth, a temporary blood vessel called the ductus arteriosus connects the pulmonary artery and the aorta. • This allows blood to bypass the lungs because oxygen is delivered to the fetus through the placenta and umbilical cord. • The temporary vessel normally closes within a few hours or days of birth since the lungs take over. • If it remains open (patent), some blood that should circulate through the body is misdirected to the lungs. • This defect can cause heart failure or endocarditis. In infants, it can be closed with medications. In older children and adults, plugs, coils or surgery can be used to close the vessel.

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3. Patent Ductus Arteriosus • • • •

• •

Aorta to pulmonary artery Common in prematurity, high altitude, maternal rubella females S/sx:clubbing, dyspnea, “machinery murmur” (2nd-3rd ICS) Cx:heart failure, endocarditis, pulmonary artery stasis/HPN Mx:Indomethacin, surgery

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Aortic stenosis • defect that narrows or obstructs the aortic valve opening, making it difficult for the heart to pump blood into the aorta. • Mild cases may not have symptoms initially, but they can worsen over time. • The defect can cause heart enlargement, left-sided heart failure, arrhythmias, endocarditis and fainting. • Treatment includes surgical repair or replacement of the valve or, in young children, widening through balloon valvuloplasty MLNG CELESTE,RN, MD

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Tetralogy of Fallot • This defect is a combination of four (tetralogy) congenital abnormalities. The four defects typically are ventricular septal defect (VSD), pulmonary stenosis, a misplaced aorta and a thickened right ventricular wall (right ventricular hypertrophy). • They usually result in an insufficient amount of oxygenated blood reaching the body. • Complications of tetralogy of Fallot (fuh-LOE) include cyanosis — sometimes called "blue baby syndrome," since the lips, fingers and toes may have a bluish tinge from lack of oxygen — as well as poor eating, inability to tolerate exercise, arrhythmias, delayed growth and development, and stroke. • Surgical repair of the defects is required early in life. • Tet spells- irritability, pallor and blackouts or convulsions,

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CYANOTIC HEART DISEASE 1. Tetralogy of Fallot • S/sx: cyanosis, clubbing, dyspnea, fatigue, squatting, “Tet spells”, failure to thrive, systolic murmur, polycythemia • Cx:thromboembolism,CVA • Mx: O2, knee-chest position, surgery 1-2 yo • Postop:monitor for arrhythmia Blalock Taussig: anastomose subclavian art. and pulmo art. - avoid BP and venipuncture in right arm

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Transposition of the great vessels/ arteries • With this defect, the positions of the aorta and the pulmonary artery (the great arteries) are reversed (transposed). • The aorta arises from the right ventricle instead of the left and the pulmonary artery arises from the left ventricle instead of the right. • This creates a circulatory pattern that prevents nourishing oxygenated blood from reaching the body. • This condition would quickly be fatal to a newborn except it's generally accompanied by another defect — commonly a septal defect or patent ductus arteriosus — that does allow oxygen-rich blood to get to the body. Surgical repair is usually necessary shortly after birth.

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2. Transposition of the Great Vessels • Aorta from R ventricle • Pulmonary a. from L ventricle • Males • S/sx:cyanosis, murmurs • Mx:PGE for PDA, Balloon catheter to create ASD, definitive surgery 1 wk-3 mos MLNG CELESTE,RN, MD

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3. Total Anomalous Pulmonary Venous Return • Pulmonary vein drains to SVC or R atrium • PDA or foramen ovale essential • S/sx:cyanosis, fatigue • CX: R heart failure • Mx:PGE, surgery

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Coarctation of the aorta • This is a narrowing (coarctation), or constriction, in a portion of the aorta. • Coarctation forces the heart to pump harder to get blood through the aorta and on to the rest of the body. • This defect can cause several life-threatening complications, including severe hypertension, aortic aneurysm, dissection or rupture, endocarditis, brain hemorrhage, stroke, heart failure and premature coronary artery disease. • Repair is typically recommended before age 10, either by surgically removing the affected portion or widening it through balloon angioplasty and placement of a stent.

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Coarctation of the Aorta • Constriction of aorta • males • S/sx: asymptomatic HPN, irritability, headache, epistaxis, dyspnea, claudication, higher BP in upper extremities, dec femoral and distal pulses,systolic murmur • Cx:chronic HPN • Mx:surgery 2 yo • Postop: monitor abdominal pain, antihypertensives

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Ndx: Ineffective cardiopulmonary and peripheral tissue perfusion related to impaired cardiac function • Proper positioning to maintain respiration • Conserve energy to promote rest • Quiet activities and stimulation • Anticipate needs • Administer prescribed drugs

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Activity intolerance r/t effects of congenital heart defect and dyspnea • •

Rest periods Adequate nutrition small frequent feedings iron supplementation

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Pulmonary stenosis • the flow of blood from the right ventricle to the pulmonary artery is obstructed by narrowing at the pulmonary valve. • When there's an obstruction (stenosis), the right ventricle must pump harder to get blood into the pulmonary artery. • The defect may occur along with other defects, such as thickening of the muscle of the right ventricle immediately below the valve.

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• in many cases, pulmonary stenosis is mild and doesn't require treatment. But because it can cause heart failure, arrhythmias or enlargement of the right heart chambers, surgery may be necessary to repair the stenosis or replace the valve. • Special balloons to widen the valve (balloon valvuloplasty) may also be used.

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Truncus arteriosus • This is a defect in which the normally distinct pulmonary artery and aorta merge into one single great vessel (truncus) arising from the right and left ventricles. • In addition, there's usually a large ventricular septal defect, essentially turning the right and left ventricles into a single chamber. • This allows oxygenated and unoxygenated blood to mix. Too much blood may flow to the lungs, flooding them and making it difficult to breathe. • It can also result in life-threatening pulmonary hypertension.Surgery is needed to close the septal defect with a patch and to separate the pulmonary arteries from the trunk. • A conduit is placed to connect the right ventricle to the pulmonary artery. Because the conduit doesn't grow with the child, repeat surgery may be necessary over time.

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Truncus arteriosus

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Hypoplastic left heart syndrome • In this condition, the left side of the heart is underdeveloped (hypoplastic), including the aorta, aortic valve, left ventricle and mitral valve. • As a result, the body doesn't receive enough oxygenated blood. In the first few days after a baby is born, the ductus arteriosus remains open (patent), allowing normal circulation, so the baby may seem fine initially. • But when the ductus arteriosus naturally closes, signs and symptoms begin, including a bluish cast to the skin from lack of oxygen, difficulty breathing and poor feeding. This condition may be accompanied by an atrial septal defect. • Treatment options for this life-threatening condition are a heart transplant or a multistage surgical procedure done during the first few years of life.

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Hypoplastic left heart syndrome

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Atrioventricular canal defect • This is a combination of defects, including a large hole in the center of the heart and a single common valve instead of the separate tricuspid and mitral valves. • Also called atrioventricular septal defect, this defect is classified by whether it's only partial, involving only the upper chambers of the heart, or complete, in which blood can travel freely among all four chambers of the heart. • Both forms allow extra blood to circulate to the lungs, causing the heart to enlarge.The condition is often associated with Down syndrome. Infants may also have trouble breathing and not grow well. • Surgery is often done in infancy to close MLNG CELESTE,RN, MD 148 the hole and reconstruct the valves.

Atrioventricular canal defect

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Other Cardiovascular Problems

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Kawasaki Disease/ Mucocutaneous Lymph Node Syndrome • Before puberty, peaks 4 yo • S/sx:spiking fever x 5 days, bilateral conjunctivitis, reddened pharynx, dry lips, strawberry tongue, cervical lymphadenopathy, peripheral edema, erythema and desquammation, truncal rash, arthritis • Patho: Respi infection immune complex vasculitis aneurysm and MI

systemic

• Dx:clinical • Mx: Salicylates and Immunoglobulins MLNG CELESTE,RN, MD

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•

Risk for ineffective peripheral tissue perfusion related to inflammation of blood vessels - Observe for chest pain, color changes, vomiting

•

Pain r/t swelling of lymph nodes and inflammation of joints - Comfort measures, administer pain medications

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Other Measures: Protect edematous areas Record intake and output Offer soft food Administer prescribed medication

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Rheumatic Fever • • • • •

Autoimmune Grp A Beta hemolytic strep 6-15 yo, peaks 8 yo 1-3 wks after untreated infection Dx: 5 major criteria – polyarthritis, carditis, subcutaneous nodules, erytHema marginatum, sydenham’s chorea minor – fever, polyarthralgia, hx of RF, inc ESR, antecedent strep infection • To diagnose, either 2 major or 1 major and 2 minor present • Cx:mitral valve insufficiency and myocarditis

J.

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Mgmt: salicylate, penicillin Ndx: Risk for noncompliance r/t knowledge deficit about importance of long term therapy - prevent initial and recurrent attacks Decreased cardiac output r/t disease process - bed rest, comfort and appropriate activities MLNG CELESTE,RN, MD

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Nursing Care • Monitor vital signs • Provide adequate nutrition • Promote safety to prevent chorea related injuries

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Gastrointestinal Disorders

Altered Gastrointestinal Functioning Acquired and congenital conditions of the GI tract can result in altered GI functioning. A thorough assessment is necessary for prompt treatment. MLNG CELESTE,RN, MD

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Interventions are geared toward: • controlling and correcting the problem • promoting adequate nutrition and normal function and • preventing complications

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Characteristics of the pediatric GI System • Peristalsis occurs within 2 ½ to 3 hours in the neonate and extends to 3 t0 6 hours in older infants and children. • The stomach capacity of the neonate is 30 to 60 ml, which gradually increases to 200 to 350 ml by age 12 months and 1,500 ml in the adolescent. MLNG CELESTE,RN, MD

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• Up until 4 to 8 weeks, the neonatal abdomen is larger than the chest and the musculature is poorly developed. • The sucking and extrusion reflexes persist until 3 to 4 months. • Saliva production begins at age 4 months and aids in the process of digestion.

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• Spit-ups are frequent in the neonate because of the immature muscle tone of the lower esophageal sphincter (LES) and the low volume capacity of the stomach. • Increased myelination of nerves to the anal sphincter allows for physiologic control of bowel function, usually at about age 2.

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• The liver’s slow development of glycogen storage capacity makes the infant prone to hypoglycemia. • From ages 1 to 3, the composition of intestinal flora becomes more adultlike and stomach acidity increases, reducing the number of GI infections.

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Diagnostic Tests

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Barium or Gastrografin Swallow Primarily used to examine the esophagus Gastrografin and barium facilitate imaging through Xrays, but Gastrografin is less toxic if it escapes from the GI tract Nursing Interventions: - Explain the procedure to the child and his parents. - Maintain the child on NPO starting at midnight before the test. - Tell the child that he must hold still during the X-ray. - After the test, monitor bowel movements for excretion of barium. Monitor GI function.

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Upper GI Imaging • Upper GI series - Swallowed Barium moves into the esophagus, stomach, and duodenum to reveal abnormalities. - Barium outlines stomach walls and delineates ulcer craters and filling defects.

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• Small bowel series - An extension of the upper GI series - Visualizes Barium flowing through the small intestine to the ileocecal valve

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NI: - Explain the procedure to the child and his parents. - Tell the child that he must hold still during the X-ray. - Make sure the lead apron is properly placed around the genital area. - After the test, monitor bowel movements for excretion of barium. - Monitor GI function.

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Barium Enema • Allows X-ray visualiztion of the colon. NI: - Explain the procedure to the child and his parents. - Usually, the child will follow a liquid diet for 24 hours before the test. - Bowel preparations are administered before the examination. - Tell the child that X-rays will be taken on a test table and that he must hold still. - Cover the genital area with a lead apron during X-ray. MLNG CELESTE,RN, MD

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Stool Specimen Examined for suspected GI bleeding, infection, or malabsorption Tests include the Guaiac test for occult blood and microscopic tests for ova, parasites and fat NI: - Obtain the specimen in the correct container. - Be aware that the specimen may need to be transported to the laboratory immediately or placed in the refrigerator. MLNG CELESTE,RN, MD

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Fiber-optic Testing • Esophagogastroduodenoscopy – a fiber-optic scope is inserted to allow direct visual inspection of the esophagus, stomach and duodenum • Proctosigmoidoscopy – the rectum and distal sigmoid colon are inspected directly • Colonoscopy – the descending, transverse and ascending colon are inspected directly

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• NI: - Explain the procedure to the child and his parents. - Obtain written, informed consent. - A mild sedative may be administered before the examination. - The child may be kept on NPO starting at midnight before the test. - The child may be placed on a liquid diet for 24 hours before the examination or require enemas and laxatives until clear.

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Endoscopic Retrograde Cholangiopancreatography (ERCP) Contrast media is injected into the duodenal papilla to allow radiographic examination of the pancreatic ducts and hepatobiliary tree NI: - Before the procedure: Explain the procedure to the child and his parents. Obtain written, informed consent. Check the child’s history for allergies to cholinergics and iodine. Administer a sedative and monitor the child for the drug’s effect.

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- After the procedure: Monitor the child’s gag reflex (the child remains on NPO status until his gag reflex returns) Protect the child from aspiration of mucus by positioning the child on his side. Monitor the child for urine retention.

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GI Intubation To empty the stomach and intestine To aid in the diagnosis and treatment To decompress obstructed areas To detect and treat GI bleeding To administer medications or feedings

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NI: Maintain accurate intake and output records - Record the amount, odor and consistency of gastric drainage every 4 hours. - When irrigating the tube, note the amount of normal saline solution instilled and aspirated Check for fluid and electrolyte imbalances. Provide good oral and nasal care. Make sure the tube is secure but that it isn’t causing pressure on the nostrils. MLNG CELESTE,RN, MD

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To support the tube’s weight and prevent its accidental removal, anchor the tube to the child’s clothing. After removing the tube from a child with GI bleeding, watch out for signs and symptoms of recurrent bleeding. Maintain a calm and reassuring manner and provide emotional support because many children panic at the sight of the tube.

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Cleft Lip and Palate •

failure of fusion of maxillary and median nasal process

•

hereditary

•

unilateral/bilateral

•

males

Mx: surgery – Cheiloplasty; usually 1-3 mos •

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Cleft Palate • midline opening of palate • usually w/ Cleft lip • Female • surgery 6-18 mos - allows anatomic changes; <1 y/o (ie, formation of palatine arch and tooth buds)

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Ndx: • Risk for imbalanced nutrition, less than body requirements r/t feeding problems • Risk for ineffective airway clearance r/t oral surgery • Risk for infection during post op period

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Nursing Care Preop •

Adequate nutrition; sips of fluid btw feeding in upright position; use rubber tipped syringe; Burp

•

Prevent ear and upper respiratory tract infection

•

Address body image and speech concerns

•

Reassurance to parents

Postop •

monitor respiratory distress d/t edema, hemorrhage

•

Suction mucus and blood gently

•

dropper feeding 1st 3 weeks; regular feeding after

•

Anticipate needs and position to prevent tension on sutures •

Position side lying in cleft lip; prone in cleft palate MLNG CELESTE,RN, MD

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Tracheoesophageal Fistula

• Communication of the esophagus and trachea • maternal hydramnios and prematurity • S/sx:coughing, choking, cyanosis, dyspnea, excessive secretion, abdominal distention • Dx: Ba swallow

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Ndx: • Risk for imbalanced nutrition, less than body requirements r/t inability for oral intake • Risk for infection r/t aspiration or seepage of stomach contents into lungs • Risk for impaired skin integrity r/t gastrostomy tube insertion site

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Nursing care Preop •

Suction regularly

•

Elevate the head

•

Gastrostomy feeding

•

hydration

•

O2

Postop 8.

Observe for respiratory distress

9.

Proper positioning –avoid hyperextension

of neck 11. Continue suction 12. Prevent wound infection 13. Provide pacifier

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Hirschprung’s Disease • Aganglionic megacolon • Absence of parasympathetic ganglionic cells in a segment of the colon (usually at the distal end of the large intestine: rectosigmoid colon) • Lack of innervation to a bowel segment causes a lack of, or alteration in, peristalsis in the affected part • Male predominance MLNG CELESTE,RN, MD

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• Causes Familial, congenital defect Commonly exists with other congenital anomalies, particularly Down’s syndrome (trisomy 21) and anomalies of the urinary tract • Pathophysiology As stool enters the affected part, it remains there until additional stool pushes it through. • The affected part of the colon dilates; a mechanical obstruction may result.

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• Complications Severe Constipation Enterocolitis – severe diarrhea, Hypovolemic shock, Death S/sx: Constipation Ribbonlike stools Failure to pass meconium or stool abdominal distention Bile stained or fecal vomiting

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• Dx: Rectal biopsy provides definitive diagnosis by showing the absence of ganglion cells Ba enema – shows distention of the colon; spasm and a narrowed lumen in the affected bowel Abdominal X-rays show distention of the colon and air-fluid levels; No air is seen in the rectum • Mx: colostomy, surgery: To decompress the colon, a temporary colostomy or ileostomy may be necessary MLNG CELESTE,RN, MD

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Ndx: •

Constipation r/t reduced bowel function

•

Imbalanced nutrition, less than body requirements r/t reduced bowel function

Nursing Care Preop 8. Administer isotonic enemas: Normal saline solution or mineral oil to evacuate the bowel Daily enemas with 0.9% NaCl Don’t administer tap water. *Tap/hypotonic water will cause cardiac congestion or cerebral edema 2. Minimal residue diet with vitamin supplementation 3. Position semi fowlers to relieve dyspnea from distended abdomen 4. pacifier MLNG CELESTE,RN, MD

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After colostomy or Ileostomy 2.

Monitor fluid intake and output (ileostomy will likely cause excessive electrolyte loss)

2.

Keep the area around the stoma clean and dry; use colostomy or ileostomy appliances to collect drainage

3.

Monitor for return of bowel sounds to begin diet

Postop 9.

Observe for abdominal distention

10. Small frequent feedings after NGT removal 11. Colostomy care 5. Assist parents to cope with children’s feeding problems MLNG CELESTE,RN, MD

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Intussusception • 2-6 mos • Invagination of intestine • S/sx:intense abdominal pain, vomiting, blood in stool “currant jelly”, abdominal distention (sausage shaped mass) • NECROSIS: fever, tachycardia, rigid abdomen • Dx:sonogram “coiled spring” • Mx:Ba enema (reduction by hydrostatic pressure), surgery

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Ndx: • Pain r/t abnormal abdominal peristalsis • Risk for deficient fluid volume r/t bowel obstruction

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Nursing Care 1.

Provide comfort measures - pacifier for infants

2.

NPO

3.

Adequate hydration via IV therapy

4.

Promote parent-infant bonding

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Pyloric Stenosis • Hypertophy/hyperplasia of pyloric sphincter • Males • S/sx:nonbillous vomiting, s/sx of of dehydration and wt loss, abdominal distention, “olive” sized mass RUQ, visible peristalsis • Dx:xray-”string sign”, USG, endoscopy • Mx: surgery MLNG CELESTE,RN, MD

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Ndx: • Risk for deficient fluid volume r/t inability to retain food • Risk for infection at site of surgical incision r/t danger of contamination from feces d/t proximity of incision to diaper area

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Nursing care •

Hydration

•

Pacifier

•

may give thickened feedings on upright position then NPO just before surgery

•

Monitor I and O, weight, and vomiting

Postop 1. dropper feeding 4-6 hrs after surgery 45 min- 1 hr duration; oral rehydration soln then half strength breastmilk/formula at 24 hr interval 2. Side lying position 3. Monitor weight and return of peristalsis 4. Wound care 5. Pacifier for oral MLNG CELESTE,RN, MDneeds

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Diaphragmatic Hernia • Herniation of intestinal content into the thoracic cavity • Left side • S/sx:respiratory difficulty, cyanosis, retractions, (-) breath sounds affected side, scaphoid abdomen • Cx: pulmonary HPN Mx:’E’ surgery MLNG CELESTE,RN, MD

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Ndx: Risk for ineffective airway clearance r/t displaced bowel Risk for imbalanced nutrition, less than body requirements, r/t NPO status

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Nursing Care Preop •

Elevate head

•

Low intermittent suction

Postop 6.

Semi-fowlers

7.

Maintain warm, humidified envt – lung fluid drainage

8.

Suction prn

9.

Chest pptx

10. NPO – prev pressure on diaphragm MLNG CELESTE,RN, MD

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Necrotizing Enterocolitis

- Bowel develops necrotic patches, interfere w/ digestion, lead to paralytic ileus, peritonitis and perforation - D/t ischemia sec shock or hypoxia, infection, immature GIT immune protection S/sx: abdomen tense and distended, stool (+) for occult blood, > 2 ml gastric residual, bradycardia, apnea Dx: abdominal xray Mgmt: d/c feeding, IV or TPN, Antibiotic, colostomy, surgery for perforation MLNG CELESTE,RN, MD

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Celiac Disease/Gluten sensitive enteropathy/Celiac sprue • Characterized by poor food absorption and sensitivity or intolerance to gluten (a protein found in grains : wheat, rye, oats, barley) • Usually becomes apparent between ages 6-18 months • Causes: Gluten intolerance Immunoglobulin deficiency MLNG CELESTE,RN, MD

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• Pathophysiology A decrease in the amount and activity of enzymes in the intestinal mucosal cells causes the villi of the proximal small intestine to atrophy and decreases intestinal absorption. • Complications Lymphoma of the small intestine

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• S/sx: steatorrhea (fatty stools) because of inability to absorb fat chronic diarrhea anorexia malnutrition; weight loss failure to thrive coagulation difficulty from the malabsorption of fatsoluble vitamins irritability anemic (Fe deficiency) abdominal pain and distention

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• CELIAC CRISIS: acute vomiting and diarrhea • Dx:clinical; IgA antigliadin Ab, gluten free diet • Mx: gluten free diet, vitamin & Fe supplementation • NDx: Imbalance nutrition: Less than body requirements r/t impaired absorption

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NI: - nutrition counseling Eliminate gluten from the diet: avoid cereals, bread, cake, cookies, spaghetti, pizza, instant soup, some chocolates Give the child: corn and rice products, soy and potato flour, breast milk or soy- based formula, and fresh fruits. Replace vitamins and calories; give small, frequent meals. Monitor for steatorrhea- its disappearance is a good indicator that the child’s ability to absorb nutrients is improving. MLNG CELESTE,RN, MD

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Endocrine Disorders

Cystic Fibrosis • Autosomal recessive • Dysfunction of exocrine glands; inc viscosity of mucous glands • Affects lungs, pancreas, intestines and sweat glands • Cystic fibrosis (multiple organ disease; the primary pathophysiologic mechanism in cystic fibrosis mucus buildup in the lungs and pancreas; steatorrhea; azotorrhea) • S/sx: GI - meconium ileus, rectal prolapse, steatorrhea, malnutrition, failure to thrive Respiratory – recurrent infection,cough, dyspnea, clubbing, cyanosis MLNG CELESTE,RN, MD

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Sweat Gland – ”salty taste” Other – males infertile, abnormal glucose tolerance Dx: history, sweat test (Cl 2-5x the normal), absence of pancreatic enzymes, immunoreactive trypsinogen in blood and pulmonary involvement Mx: chest physiotherapy, antibiotics, pancreatic enzymes, vitamins, lung or pancreas transplant

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Ndx: • Imbalanced nutrition, less than body requirements r/t inability to digest fats • Ineffective airway clearance r/t inability to clear mucus from the respiratory tract

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Nursing Care 1. 2. 3. 4. 5.

Hi calorie, Hi CHON, moderate fat diet Nebulization and physiotherapy Frequent Position changes when in bed Oral care Adequate rest and comfort

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Musculoskeletal Disorders

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Clubfoot (Talipes) • Ankle-foot disorders • Types: Varus – inward rotation Valgus – outward rotation Calcaneous – upward rotation or dorsiflexion Equinas – downward rotation or plantarflexion

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• Males • Unilateral more common • Talipes Equinovarus (plantar flexion and medial deviation) – 95% • S/sx: foot cannot be manipulated by passive exercises into correct position • Mx: cast and splint, surgery then casting and corrective shoes MLNG CELESTE,RN, MD

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NDx: Risk for impaired skin integrity r/t corrective devices Nursing Care • Exercise • Cast and brace care • Skin care • Restraints if necessary • Diversional activities • Health teaching

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Congenital Hip Dysplasia •

• • •

Imperfect hip development affecting femoral head and acetabulum Female Unilateral more common Inc frequency w/ breech delivery

A. Lower right leg B. asymmetric skin

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S/sx: • limited abduction of affected hip • shortening of leg on affected side (Galeazzi/Allis sign) • asymmetric thigh and gluteal folds • buttocks on affected side will flatten on prone • pelvis dips on normal side when standing on affected leg (Trendelenburg) • palpable click (Ortolani’s click) • Mx: maintaining hip in abduction traction and casting

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A. Ortolani’s test B. Barlow’s Test

A. Frejka splint B. Pavlik Harness C. Hip abduction for subluxation MLNG CELESTE,RN, MD

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NDx: Impaired physical mobility r/t immobilization device Nursing Care • Maintain proper positioning-keep legs abducted • Adequate nutrition • Diversional activities • Regular exercise • Ensure adequate circulation • Provide comfort • Maintain cast, traction, splint

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Neurological Disorders

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Brain Tumor • • • •

Second most common form of cancer in children 1-10 yo, peak 5 yo Usual location - brainstem or cerebellum TYPES: Astrocytoma- glial tissue; 5-8 yo Medulloblastoma – cerebellum; 5-10 yo; cause compression of 4th ventricle; most common Brain stem tumor – support tissue of neural cells; paralysis of 5th-7th, 9th-10th CN

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S/sx: headache, vision changes, vomiting, enlarging head circumference, papilledema, lethargy, ataxia, nystagmus, personality changes, seizures, lethargy, coma Dx: skull films, bone scan, CT scan, Lumbar puncture, MRI, angiography Mx: surgery, chemotx

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2. 3. 4. 5. 6.

Preop Stool softeners Dexamethasone Dilantin Shave head Prepare patient and family psychologically

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Postop 2. Proper position – low fowler’s 3. Anticipate needs 4. Saline eye drops as needed 5. Monitor VS and NVS 6. Regulate IV 7. Observe head dressings 8. Provide comfort and opportunity for self expression

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Benign Febrile Seizure • • • • • • •

6 mos - 5 years fever >/= 38.5C generalized tonic-clonic rarely persist > 10 minutes Postictal stage 30-40 % recurrence (+) family history

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NURSING CARE • • • • •

Turn child to side & allow to drool Do not restrain Do not put anything in the mouth Dec temp Refer if: sx persist another sx occurs delirious/difficult to rouse after sx

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Seizure

• Paroxysmal involuntary disturbance of brain function Epilepsy: recurrent seizures unrelated to fever or to acute cerebral insult

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Generalized Seizures • Absence Seizures/Petit Mal: - staring spell that lasts a few seconds - 100x a day - appear as if daydreaming • Generalized/Grand Mal/Tonic-Clonic 4 stages Prodrome: mood or behavioral changes; precede attack by hours Aura: immediately before seizure and will localize the attack to its point of origin within the nervous system MLNG CELESTE,RN, MD

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Tonic phase (10 secs) eyes open. Elbows flexed. Arms pronated. Legs extended. Teeth clenched. Pupils dilated. Breath held- cyanosis. Bowel or bladder control may be lost at the end of this phase Clonic phase (1-2 min) tremor gives way to violent generalized shaking. Eyes roll backwards and forwards. Tongue may be bitten, tachycardia develops. Breathing commences at the end of phase.

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• Partial Seizures Simple partial: with motor and sensory symptoms Complex partial/psychomotor: simple partial developing into a complex seizure, consciousness impaired at onset Partial seizure with secondary generalization discharges spread from their point of origin and excite other structures

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Mx: Acute Attack • Provide O2 • Position properly • Turn head to side • Promote safety • Do not restrain

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Prevention • Health teaching • Supervise activities • Antiepileptics Generalized Tonic-Clonic, Focal – Phenobarbital, Phenytoin Absence – Ethosuximide, Valproate

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Erb Duchenne’s Paralysis

• Damage on C5-C6 due to birth trauma • Unilateral • S/sx: shoulder adducted, internally rotated, elbow extended, forearm pronated and the wrist flexed “waiter’s tip”, (-) moro reflex • Mx: PT, surgery • Nsg care: advise exercise and frequent follow up

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Cerebral Palsy • Damaged motor function d/t anoxic brain injury secondary to infection, perinatal asphyxia, metabolic disorder • Nonprogressive • TYPES: Spastic Dysphagia – most common; hypertonicity Athetoid/dyskinetic – worm-like Ataxic – wide based gait w/ repetitive mvmt Mixed MLNG CELESTE,RN, MD

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• S/sx: spasticity/rigidity, difficulty feeding, delayed speech and motor devt, mood swings, impulsive, short attention span, irritable • Dx: Clinical • Mx: antispastics, antibiotics, surgery, nutrition, prevention of injury, PT NDx: Risk for injury r/t neuromuscular impairment Self care deficit r/t neuromuscular impairment - ensure safety, assist in ADL, ensure adequate nutrition, assist children achieve maximum potential MLNG CELESTE,RN, MD

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Hydrocephalus •

Impaired circulation and absorption of CSF 2 TYPES: 3. Obstructive/Noncommunicating – w/n ventricular system 4. Nonobstructive/Communicating – obliteration of SA cisterns or malfunction of arachnoid villi S/sx: head enlargement, ant fontanel wide and bulging, scalp veins dilated, broad forehead, sclera shows above iris, brisk tendon reflexes, spasticity, irritability, lethargy, poor appetite, cracked pot sound on percussion MLNG CELESTE,RN, MD

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Ndx: Risk for ineffective cerebral tissue perfusion related to increased intracranial pressure - monitor vs, head circumference, I and O - O2 - position flat or head 30 deg - do not lie on operative site

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Risk for imbalanced nutrition, less than body requirements, related to increased intracranial pressure - NPO until bowel sounds return - position head w/o flexion - observe for constipation - note how child sucks - IV fluids - obtain daily weight

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Spina Bifida • Collective term for all SC disorders Spina Bifida Occulta – posterior laminae fail to fuse - dimpling, abnormal tufts of hair Meningocoele - meninges herniate through unformed vertebrae; protrusion covered by a layer of skin - usually occurs in the lumbar region - protrusion is covered by a skin layer or only the clear dura mater MLNG CELESTE,RN, MD

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Myelomeningocoele – SC and meninges protrude through the vertebrae defect - absent motor and sensory function - flaccidity, lack of sensation in LE - loss of bowel and bladder control - may be accompanied by hydrocephalus Dx: sonography

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• Risk for infection r/t rupture or bacterial invasion of the neural tube sac - position side lying or prone - keep sac moist - place under radiant warmer - post op – prone until site is healed • Risk for ineffective cerebral tissue perfusion r/t increased intracranial pressure - measure head circumference - assess for s/sx of inc ICP

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• Risk for impaired skin integrity r/t required prone positioning - reposition head every 2 hrs if w/ hydrocephalus - change diapers frequently • Impaired physical mobility r/t neural tube d/o - passive exercises - may use leg braces, crutches - inspect lower extremities and buttocks for irritation or possible infection • Risk for impaired elimination r/t neural tube d/o - intermittent catheterization - surgery MLNG CELESTE,RN, MD

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Meningitis • Inflammation of meninges • Bacterial, Tuberculous, viral S/sx: opisthotonus, neck rigidity, irritability, high pitched cry Dx: Lumbar puncture, Bld C/S, Ct scan, MRI NDx: Risk for infection r/t presence of infective organism Tx: Abx

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Hematologic Disorders

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Iron Deficiency Anemia Infant’s iron supply 4-6 mos Anemia – 9-24 mos S/sx: pallor, tachycardia, irritability, Hg < 9 g/dl, susceptible to infection Mgmt: iron fortified formula, iron rich diet and vitamins Nsg care: give iron w/ vit c, use w/ dropper at the back of the mouth, expect black stools, provide iron rich food MLNG CELESTE,RN, MD

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Hemophilia A • Inherited interference w/ blood coagulation • Factor VIII • Sex linked recessive • S/sx: excessive bleeding NB – apparent Pre-school – accidents/falls School age – bleeding between joints Adolescent – ulcers, hematuria » Mx:factor 8 MLNG CELESTE,RN, MD

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NDx: Acute pain r/t bleeding into joints Nursing Care • • • • •

Promote safety Watch out for bleeding – rest area, ice compress, elevate body part Monitor transfusion reaction Passive ROM Assist in gaining control of situation

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Acute Lymphocytic Leukemia •

Uncontrolled proliferation of WBC

•

Lymphoblast

•

Most frequent CA in children

•

2-16 yo

•

Males

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S/sx: pallor, low grade fever, lethargy, petechiae, bleeding, vomiting, anorexia, bone pain, painless lymphadenopathy Dx:WBC variable w/ blasts, low platelet and hematocrit, anemia BMA > 25% blast cells

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Mx: chemotherapy Cx: CNS s/sx, renal failure NDx: Risk for infection r/t decreased immune function Activity intolerance r/t reduced oxygen carrying capacity of blood

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Nursing Care • • • • •

Prevent infection monitor bleeding and transfusion reactions Provide comfort and pain alleviation Health teaching Emotional and psychological support

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Beta Thalassemia • • • •

B chain defect Heterozygous – Thalassemia minor Homozygous – Thalassemia major 4-6 mos old S/sx: anemia Dx: peripheral blood smear Tx: blood transfusion - pRBC

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Genitourinary Disorders

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Urinary Tract Infection • • • •

Females E coli Ascending infection S/Sx:infants – mimic GI d/o; dysuria, frequency, hematuria, low grade fever, abdominal pain and bedwetting • Dx: urine culture suprapubic any amount clean catch > 100,000/ml • Mx: antibiotic hydration MLNG CELESTE,RN, MD

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Acute Glomerulonephritis • • • •

Inflammation of glomeruli or kidney Follows infection with strep10-14 days 5-10 yo Males

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S/sx:sudden onset of edema and hematuria, proteinuria, hypertension Dx: urinalysis and 24 hour urine hypoalbuminemia inc ESR, BUN, Crea, antistreptolysin O

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Mx: semi fowlers diuretics, antibiotics O2 antihypertensives Nsg Care: quiet play activities diet – normal CHON, mod salt restriction, fluid restriction daily weight and output

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Nephrotic Syndrome • Altered glomerular permeability(autoimmune); inc permeability to albumin • 3 yo • Males • Minimal change syndrome S/sx: proteinuria, edemaperiorbital area, hypoalbuminemia, hyperlipidemia

24

Dx:urinalysis and hr CHON, inc ESR

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Mx:steroids, immunosupressant NDx: Risk for decreased fluid volume r/t CHON and fluid loss Imbalanced nutrition: less than BR r/t CHON and fluid loss Nsg care: Adequate nutrition, proper diet – dec salt Weigh daily, monitor I and O Protect edematous areas Administer prescribed drugs Health teaching MLNG CELESTE,RN, MD

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Wilm’s Tumor • • • • •

Malignant tumor of the kidney Associated with other anomalies 6 mos-5 yo, peaks 3-4 yo Good prognosis S/sx:abdominal mass, hematuria, low grade fever, anemia, wt loss • Dx: CT scan • Mx: Nephrectomy, radiotherapy avoid abdominal palpation

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Hypospadia/Epispadia

A. Hypospadia

B. Epispadia

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CONGENITAL MALFORMATIONS OF THE URINARY TRACT

• Epispadias -Urethral opening on the dorsal surface of the penis • Surgical correction

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• Hypospadias -Male urethral opening on the ventral surface of penis, or female urethral opening in vagina • Surgical reconstruction

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Hypospadia - common - chordee - fibrous band Mx: surgery Nsg care: Post op – pain relief assist parents in coping

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Cryptorchidism • Failure of one or both testes to descend • Descend up to 6 weeks at birth • May be d/t dec testosterone • S/sx:right testis more common • Mx:chorionic gonadotrophin hormone Orchiopexy 1 yo

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Other Diseases

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Atopic Dermatitis • 2 mos-3 yo • R/t food allergy S/sx: papular and vesicular skin eruptions w/ erythema, pruritus, dry,flaky scales upon healing Mx: reduce allergen, topical steroids NDx: Impaired skin integrity r/t eczematous lesion Nsg care: Reduce allergen Prevent skin dryness and pruritus MLNG CELESTE,RN, MD

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Otitis Media • • • •

Inflammation of the middle ear 6-36 mos, 4-6 yo S. Pneumoniae, H. Influenzae, M. Catarrhalis Follows URTI S/sx:pain in affected ear, fever Cx:hearing impairment Mx: antibiotics, analgesics, antipyretics NDx: Acute pain r/t inflammatory process - provide comfort, reinforce completion of antibiotic, offer liquids and finger food (prevent pain when chewing) MLNG CELESTE,RN, MD

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Inguinal Hernia • Protrusion of a bowel through the inguinal ring • males • S/sx:painless lump in the groin • Cx:bowel strangulation • Mx:surgery <1 yo • Post op Nursing care: wound care assess circulation in the leg

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diarrhea

• Viral – Rotavirus, Adenovirus • Bacterial – Shigella, Salmonella, Cholera • Protozoan – Amoeba • TYPES: • Mild: fever, irritable, 2-10 episodes/day, dry mucous membranes, tachycardia • - 2.5-5% wt loss • Mx: oral rehydration

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Severe: fever, tachycardia, tachypnea, pale and cool skin, apprehensive/lethargic, obvious s/sx of dehydration, UO scanty, several episodes of loose stools -5-15% wt loss • Cx:dehydration • Mx:fluid and electrolyte replacement • Dx:Stool exam and culture Electrolyte determination NDx: deficient fluid volume r/t fluid losses in stools - promote hydration and comfort, Record I and O

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Protein Energy Malnutrition Marasmus • Low calorie, low CHON • 0-2 yo • (-) edema • “all skin and bone” • (+) growth retardation • Apathetic, quiet • Good appetite • Infrequent skin/hair changes • Anemia uncommon MLNG CELESTE,RN, MD

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Kwashiorkor Low CHON 1-3 yo (+) edema Wasting variable Growth retardation variable Irritable, moaning Poor appetite (+) skin and hair changes (+) anemia

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Poisoning Usually 2-3 yo Soap, cosmetics, detergents, drugs Males>females – children Females>males – adolescent Mx: 7. Determine age & wt, type of poison swallowed, time of ingestion, route of poisoning, amt ingested, present condition of child

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2.Use syrup of ipecac to induce vomiting except: caustic, corrosive, hydrocarbon, px is comatose 3. Activated charcoal then syrup of ipecac 4. Monitor VS 5.refer to hospital/poison control center

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POISONING Acetylsalicilic acid (Aspirin) S/sx:nausea, vomiting, fever, profuse sweating, flushing, hyperthermia, hyperventilation, convulsions, coma Mx:stabilize airway and breathing Fluid and electrolyte replacement Activated charcoal Alkalinization of urine hemodialysis

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Burns Assessment A airway - check nose, face and neck (priority) singed and sooty hair of the nose, give high flow 02 B breathing C circulation - if there is no breathing and circulation start CPR D check for disability and manage accordingly E expose or determine extent of injury

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Kinds of Burn: Flame – gasoline, kerosene, petroleum Chemical – acid or alkali Electrical – most fatal Radiation – sunlight < 1 yo – scalding Pre school – reaches up a stove, spills coffee Older children – flame burns

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TBSA Parkland Formula (4ml x TBSA x BWkg) 1st 8H give ½, 2nd 8H give ¼ and for the 3rd 8H give the last part)

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0-1 yo Head Neck Ant trunk Post trunk r. Buttock L buttock Genitalia r. Upper arm l. Upper arm

15 yo

19 2 13 13 2.5 2.5 1 4

1-4 yo 5-9 yo 10-14 yo 17 13 11 2 2 2 13 13 13 13 13 13 2.5 2.5 2.5 2.5 2.5 2.5 1 1 1 4 4 4

4

4

4

4

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9 2 13 13 2.5 2.5 1 4

289

0-1 yo 1-4 yo 5-9 yo 10-14 yo 15 yo r. Lower arm

3

3

3

3

3

l. Lower arm

3

3

3

3

3

r. Hand

2.5

2.5

2.5

2.5

2.5

l. Hand

2.5

2.5

2.5

2.5

2.5

r. Thigh

5.5

6.5

8

8.5

9

l. thigh

5.5

6.5

8

8.5

9

r. Leg

5

5

5.5

6

6.5

l. leg

5

5

5.5

6

6.5

r. Foot

3.5

3.5

3.5

3.5

3.5

l. foot

3.5

3.5 3.5 MLNG CELESTE,RN, MD

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3.5 290

Classification 1st degree – partial thickness (painful, no blisters, erythematous); epidermis 2nd degree – deep partial thickness (painless, with blister, blanch); epidermis and dermis 3rd degree – full thickness (painless, leathery, whitish/charred) 4th degree – bones and visceral organs are affected

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-

Hospitalization recommended Total burns > 10% of BSA or > 2% FTB *halved if < 2 yrs old Hands, feet, face or genitalia involved Inhalation injury Associated injuries present Burn is inflicted Infected burn Prior medical illness comatose

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Management: - 1st 24 hrs - D5LR - 8-24 hrs post burn - colloid - Give TIG or TAT and TT - Prophylactic antibiotic - Sterile dressing for wound Small minor burns – dressings, soap and water More extensive burns – antibacterial cream/ointment, thick/thin dressings and cleansing bath

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Down’s Syndrome Trisomy 21 Maternal age >35 yo, paternal age >55 Features:nose is broad and flat, eyelids have an extra fold of tissue at the inner canthus, palpebral fissure slants upwards, white specks in iris, tongue protrudes, back of the head is flat, neck is short, extra fat pad, low set ears, poor muscle tone, short thick fingers, simian crease, cognitively challenged

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Poor immune function Congenital heart diseases Cataract ALL occurs 20x more Mx: early education and play opportunities prevention of infection counseling and support

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Child Abuse • Non accidental injury inflicted by an adult • Physical, emotional, sexual • Children grow up to be abusers themselves and are violent • Nsg Resp: treat injury, report to DSWD, NGO, Bantay Bata

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Substance Abuse

• • • • • •

Use of chemicals Improve mental state Induce euphoria Peer pressure Feel more confident Adolescent rebellion

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TYPES OF ABUSED SUBSTANCES

Tobacco - sign of maturity Alcohol - most frequent - no stigma Amphetamines – sense of well being, alertness, self esteem and wt loss

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TYPES OF ABUSED SUBSTANCES Anabolic steroids – improves athletic ability Marijuana – stress reliever bec gives a sense of well being, altered sensory perception

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TYPES OF ABUSED SUBSTANCES

Hallucinogens – distortions in vision, smell or hearing Opiates – physiologic craving Cocaine – inc VS, dec appetite, cardiovascular arrest, euphoria, excitement, hallucinations

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ASSESSMENT OF SUBSTANCE ABUSE • • • • • • •

Failure to complete homework Poor reasoning ability Decreased school attendance Frequent mood swings Deteriorating physical appearance Recent change in peer group Negative perception of parents

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Nursing Intervention

• Caution against drug abuse • Provide counseling • refer to rehab facilities

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Thank you!

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