Pediatric CCRN: Pulmonary System

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Manually Assisted

- manually compressing lower thorax and abdomen or by binding the abdomen theoretically improves cough efficiency. (used in kids with weak abdominal muscles or neuromuscular weakness)

Autogenic Drainage

A variation on deep breathing for patients with thick secretions that are difficult to raise. Examples CF or severe COPD. Technique 3 phases first : unstick patient breathes out completely and then takes slow deep breaths and exhales fully several times, suppressing urge to cough. This loosens mucus in lower airways Next: Collect pt takes 10 to 20 slightly deep breathes, exhaling normally, still suppress cough. This helps mucus move up to middle airways. Last: Evacuate the patient takes 10 to 20 breaths and huff cough to move mucus up and out.

Beta-Agonist Medications

Albuterol, levalbuterol Description: - dilation of the airways via administration of a beta 2 agonist, found in smooth muscle of airways Indications: - relief of acute bronchospasm - management of asthma exacerbation NSG Considerations: - adverse effects: tachycardia, tremors, headache, nausea, sleep disturbances. - monitor KCl levels, O2, HR, PFTs, RR, WOB, and blood gases.

Blood Gas Values

Arterial pH 7.35-7.45 pCO2 = 35 - 45 mmHG pO2 = 80 - 100 mmHg HCO3 = 22 - 26 mEq/L Base excess +2 to -2 O2 Sats 95-97% Mixed Venous pH 7.31-7.41 pCO2 = 40 - 50 mmHG pO2 = 80 - 100 mmHg HCO3 = 22 - 26 mEq/L Base excess +2 to -2 O2 Sats 70-75% Capillary pH 7.35-7.45 pCO2 = 35 - 45 mmHG pO2 = less than arterial HCO3 = 22 - 26 mEq/L Base excess +2 to -2 O2 Sats less than arterial

Remedial Agents: Bradycardia or Bronchospasm

Atropine Description: - anticholinergic agent antagonize acetylcholine which produces central vagal blockade (tachycardia, increased BP, mitosis) - can cross the BBB and cause CNS stimulation - consider use in pts with asthma. Indications: - premedication for meds known to cause bradycardia, decreased saliva, bronchodilation and symptom control while using a beta-blocker. NSG Considerations - pupil size and responsiveness are altered. Fixed dilated pupils should not occur. - side effects bothersome to awake pts ( increased HR, dry mouth, and CNS effect {delirium, agitation}) Bradycardia Dose: 0.01-0.02 mg/kg Min: 0.1 mg Max: 1 mg Bronchospasm Dose: 0.05 mg/kg in 2.5 mL NS Q6h via MDI or Neb. Monitor for muscarinic effect: bradycardia, salivation, bronchospasm.

Arterial Oxygen Content

CaO2 = (Hb x 1.34 x SaO2) + (0.003 x PaO2) Normal: 20 mL/dL

Alfentanil

Children <12 years: limited dosing data. Contraindications: hypersensitivity Comments: monitor resp, CV, neuro status for degree of analgesia/anesthesia.

Acute pulmonary embolism

Definition: Pathophysiology: Clinical Presentation: Patient Care Management: Outcome:

Airway Obstruction

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Asthma Acute/Chronic

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BPD/chronic lung disease of prematurity

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Environmental tobacco smoke exposure

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Foreign body aspiration

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Obstructive Sleep-Disordered Breathing

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Pediatric Acute Respiratory Distress Syndrome

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Pulmonary hemorrhage

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Pulmonary hypertension

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Thoracic Trauma

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aspiration pneumonia

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cardiopulmonary resuscitation (CPR)

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cystic fibrosis (CF)

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pulmonary edema

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respiratory failure

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smoke inhalation injury

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tracheomalacia

Definition: 1. abnormal collapse of trachea d/t local or generalized weakness of tracheal wall leading to respiratory obstruction. 2. commonly acquired from ETT in premature infant 3. primary and secondary forms and either can be congenital. 4. Primary; an intrinsic abnormality of the tracheal wall. 5. Secondary; extrinsic compression of the tracheal wall. 6. Congenital; usually associated with cardiovascular abnormalities (Double aortic arch, Anomalous innominate artery, and pulmonary artery sling) 7. Also associated with bronchomalacia and other tracheal abnormalities like TEF or laryngeal cleft. Clinical Presentation: 1. manifestions vary and dx via endoscopy 2. likely a stridor, usually expiratory 3. usually present in the immediate neonatal period Patient Care Management: 1. generally self-limiting and requires no tx 2. If severe, document collapse episodes, sedation and ventilation may be needed. 3. Family CPR teaching 4. Surgical tx, vascular ring, aortopexy, tracheostomy, CPAP, segmental resection, stents, or cartilage grafting. 5. bronchodilators may worsen tracheomalacia and use with caution. Outcome: generally condition is out grown by the time the child is 2yo.

Tuberculosis

Definition: 1. 2nd cause of death of infectious disease. 2. Rates are higher in urban, low income among non-white ethnic groups. 3. Myobacterium tuberculosis. Children are susceptible to the human and the bovine organism Pathophysiology: 1. Person-Person transmission, airborne, droplets can remain suspended in the air for hours. 2. Many children do not develop disease but rather latent infection. Usually have a positive skin test and +CXR. 3. All children progress through an asymptomatic period (3-8 weeks), development of clinical disease is dependent on age younger children (under 2) are at greatest risk. After age 10 children will show adult like symptoms Clinical Presentation: may be asymptomatic or develop wide range of symptoms. - fever - malaise - anorexia - weight loss - cough - aching pain - chest tightness - hemoptysis Symptoms with disease progression - ^RR - poor lung expansion on affected side - diminished breath sounds and crackles - dullness to percussion - persistent fever - generalized symptoms - pallor - anemia - weakness - weight loss Patient Care Management: 1. Dx based on exam, hx, TB skin test, CXR, blood test, cultures. 2. TB skin test is usually positive 2-10 weeks after initial infection 3. latent TB is used to describe a positive skin test but no on going symptoms. 4. Active is used to describe pt with clinical symptoms. Goal is to sterilize lesion with drugs. (ethambutol, isoniazid, pyrazinamide, and rifampin) if drug resistant TB (amikacin, capreomycin, cycloserine, ethionamide, kanamycin, levofloxacin, para-aminosalicylic, and/or streptomycin. 5. Goal is to kill replicating TB organism Outcome:

Acute Epiglottitis

Definition: 1. SEVERE LIFE THREATENING MEDICAL EMERGENCY. progressive infection of epiglottis and surrounding areas. 2. obstructive inflammatory process that primarily occurs in children 2-5 years old but can occur from infancy to adulthood. 3. obstruction is supraglottic vs subglottic like laryngitis. 4. Usually Haemophilus influenzae (Hib), sometimes Strep. pyogene, S. pneumoniae, or S. aureus. Pathophysiology: Thickened epiglottis and aryepiglottic folds lead to narrowing of the airway and turbulent gas flow. Pulmonary edema may occur from increased transmural pressure gradient from pulmonary vasoconstriction resulting from alveolar hypoxia. Clinical Presentation: 1. Hx: shows acute onset of symptoms (high fever, sore throat, dyspnea, rapidly progressing obstruction.) Child may go to bed asymptomatic and wake with symptoms and appear sicker than pictured. 2. Physical Exam: Child wants to sit upright and lean forward with chin thrust out mouth open (tripod position), dysphagia, drooling, dysphonia, irritability, and anxiety. May appear hypoxic, red throat large edematous epiglottis. Throat exam done with emergency equipment on stand-by. 3. Presentation: abrupt fever onset, sore throat, drooling, muffled voice, and hyperextension of neck. WOB mild to moderate. Stridor is a late finding and suggests near complete airway obstruction. 4. Assess: degree of stridor, color, retractions, air entry, and LOC 5. Diagnostic Tests: avoid anxiety provoking procedures, blood cultures to identify organism after airway is secured. 6. Clinical Course: resolves in 24 to 72 hrs with ABX, most pts have cocomitant bacteremia. Patient Care Management: 1. Prevention: Hib vaccine 2. Direct Care: OR for intubation and direct laryngoscopy, keep patient comfortable, ABX of ceftriaxone, cefotaxime, or combination of ampicillin and sulbactam. Maybe steroids to help reduce edema. 3. Suportive Care: PICU admit for monitoring, sedation, muscle relaxants to prevent self-extubation. extubation criteria includes air-leak around tube. 4. Complications: intubation has fewer complications vs. tracheostomy along with shorter recovery and hospitalization. 5. Outcome: no long-term deficits, if managed appropriately.

Pertussis (whooping cough)

Definition: 1. acute respiratory infection caused by Bordetella pertussis or B. parapertussis 2. Highly contagious and particularly threatening in young infants. 3. Can result in encephalopathy, seizures, and pneumonia. 4. highest incidence in spring and summer 5. a single attack = lifetime immunity. Pathophysiology: 1. via aerosol droplets from infected to humans, adheres to ciliated upper and lower respiratory tract cells. 2. Invasiveness is rare 3. The precise mechanisms during infection are unknown. 4. Pathology causes inflammation, congestion, and infiltration of the respiratory mucosa with lymphocytes and granulocytes and leads to accumulation of viscous secretions in the bronchi, bronchial obstruction and occasional atelectasis. Clinical Presentation: 1. Catarrhal Stage: begins with symptoms of URI, sneezing, lacrimation, cough and low-grade fever. Symptoms continue for 1-2 weeks when dry hacking cough becomes more severe. 2. Paroxysmal Stage: coughing is the most prevalent symptom, more common coughing at night followed by high-pitched "whoop" cheeks become flushed or cyanotic, eyes bulge and tongue protrudes. Vomitting follows each attack, generally lasts 4-6 weeks. 3. Infants younger than 6 months may not have the characteristic whoop cough, but had trouble maintaining saturations with secretions. Patient Care Management: 1. Most can be managed at home with supportive care, but admission can occur (severe respiratory symptoms and/or apnea) 2. Preventative care: immunizations and antibiotic therapy (erythromycin, azithromycin, and clarithromycin) 3. hospitalization, increased oxygen intake and humidity, fluids, ICU management and mechanical ventilation if needed for those under 6 months. 4. complications include pneumonia, apnea (<1yr), atelectasis, PH (critical pertussis), otitis media, seizures, hemorrhages, weight loss, dehydration, hernias, prolapsed rectum, syncope, sleep disturbances, rib fractures, and incontinence. 5. Hospilization care: droplet percautions, NP swab for dx, encourage fluids, position on side for vomiting, ensure O2 sats, WOB, rarely progress to ECMO, houshold contacts should be treated with ABX, encourage vaccine, encourage healthcare workers, pregnant women and those in contact with infants to get booster shots. Outcome: 1. symptoms usually last for 6-10 weeks but may last longer. 2. mortality is higher in children under 6 months.

Bacterial Tracheitis

Definition: 1. infection of mucosa of upper trachea, features of both croup and epiglottitis. 2. see in children under 3 usually, may cause obstruction that leads to respiratory arrest. 3. rare, but most life-threatening. Pathophysiology: 1. complication of LTB with S. aureus, Moraxella catarrhalis, S. pneumoniae, and H. influenzae. Complete airway obstruction may occur as a result of copious mucopurulent secretions. 2. subglottic edema with ulceration, erythema, and psuedomembraneous formation on tracheal surface with thick secretions. 3. epiglottis and arytenoids are usually normal although epiglottis and bacterial tracheitis may coexist. Clinical Presentation: 1. History: symptoms of URI, rhinorrhea, low-grade fever, cough sore throat, hoarse voice. 2. Physical examination: rapidly mroe symptomatic with RDS, increased WOB, airway compromise, higher fever. 3. Diagnostic Tests: bronchoscopy, blood cultures, lateral neck xray 4. Clinical Coarse: many require intubation and mechanical ventilation for 3-7 days. Frequent tracheal sxn. Patient Care Management: 1. Maintenance of airway and tx of BP instability. 2. IV ABX 3. Steroids before extubation 4. may require tracheostomy, but rare. Outcome: mortality rates 18-40% with significant morbidity association including cardiopulmonary arrest, ARDS, toxic shock syndrome, septic shock, resp. failure, MODS.

pneumonia

Definition: 1. lower respiratory tract infection that causes inflammation of the lung parenchyma and is typically associated with fever and respiratory symptoms. 2. single largest cause of death in children under 5, world wide. 3. cause depends on age of pt. and underlying disease a. neonates 0-3 months: maternal flora like GBS b. infants older than 3 mo up to 5yr: over half associated with viral infection. c. school-age older than 5: mycoplasmas pneumoniae and Chlamydophila pneumoniae 4. If immunized with Hib, less likely to be infected by these. 5. Compromised host may predispose a pt: a. having a ETT or Trach b. Pulm. Aspiration from CNS injury/GERD/TEF c. abnormal airways secretion (BPD, CF, neuro devastated pts. d. underlying chronic conditions or poor nutrition e. diseases that alter the immune system. f. secondary infection after influenza. Pathophysiology: 1. organism initiates inflammatory response which causes alveolar edema. Edema is then a play where the bacteria multiplies 2. transmission through inspiration, aspiration, or systemic circulation. CXR can help determine location of pneumonia. a. lobar pneumonia: one or more lobes b. bronchopneumonia: terminal bronchioles c. interstitial pneumonia: alveolar walls, diffuse/viral Clinical Presentation: 1. Hx: mild URI or sudden fever/cough/chest pain 2. Physical Exam: a. infants present with lethargy, poor feeding, irritability. b. Nasal flaring, grunting, retractions, tachypnea, tachycardia, , possible cyanosis c. wheezing is more common in viral vs. bacteria, fine crackles, diminished d. ABD distention from swallowed air e. liver displacement due to hyperinflation of lungs f. dehydration 3. Diagnostics: a. CXR: confirms dx and indicates effusions/empyema, CXR may leg behind clinical symptoms. b. Labs: CBC w/diff, blood cultures, sputum, viral panal c. invasive testing: Quantitive bronchoalveolar lavage (BAL), lung biopsy with severe disease or immunosuppressed 4. Clinical Course: depends on pt age/course/cause, sometimes admission to ICU, complications include lung abscess, narcotizing pneumonia, pneumatocele, empyema, pleural effusion, bronchopleural fistula, pneumothorax. Patient Care Management: 1. Prevention: a. up-to-date vaccinations b. Septrs prophylaxis for immunocompromised c. treat pregnant women (GBS and Trach) 2. Direct Care a. monitor for increased repiratory distress, AMS, and cardiovascular compromise. b. maintain sats >90%, intubate and mechanically ventilate for respiratory failure. 3. Supportive Care: a. 10-20 mL/kg bolus b. ABX based on age group and organism c. further investigation if no improvement after 48-72 hrs on ABX. Outcome: 1. Respiratory Complications include pleural effusion, empyema, pneumatocele, necrotizing pneumonia, lung abcess, bronchopleural fistula and pneumothorax 2. most make uneventful recovery, children with recurrent should consult pulmonary.

Congenital Diaphragmatic Hernia (CDH)

Definition: 1/2,000-5,000 births, incomplete formation of the fetal diaphragm, usually on the left side. Anomalies associated with include neural tube defects, cardiac defects, and midline anomalies. Pathophysiology: 1. Herniation of abdominal contents into the thoracic cavity affecting fetal lung development. Compresses the lung effected and shifts the mediastinum to the opposite side and compresses the other lung resulting in varying degrees of bilateral pulmonary hypoplasia. 2. Diaphragm forms during the 8th to 10th week of fetal life. Clinical Presentation: 1. Dx usually made antenatally, from birth infants present typically with a scaphoid abdomen, funnel shaped chest, trachea and mediastinum shifted to "good lung." Depending on severity pt could appear well or have choking episodes, apnea and acute resp. failure. 2. Tachypnea and retractions 3. decreased breath sounds or absent on affected side with heart sounds shifted to the unaffected side. Patient Care Management: 1. Stabilize baby and optimize respiratory function, delayed surgical repair increasingly accepted. 2. Repair via thoracotomy or transverse abdominal approach. Reduction of herniated viscera, identification and excision of any hernia sac, and repair defect. 3. More aggressive support includes ECMO. Infants who are symptomatic within first 6 hours have highest mortality. Infants who can handle gentle ventilation are less likely to require ECMO. 4. ETT, UVC and UACs 5. Chylothorax complicating factor of CDH repair 6. post-operative FTT r/t GERD and oral dysfunction is common. 7. NSG care focuses on preventing increased PVR, avoid hypoxemia, acidosis, hypothermia, and hypoglycemia. 8. Minimal stim. environment. Outcomes: 1. overall prognosis is poor with major cause of death being pulmonary hypoplasia and/or associated anomalies. If liver is in the chest and cardiac disproportion before 24 weeks. left-sided hernias with intra-abdominal stomach after 24 weeks most favorable. 2. Survival after repair 39-95% 3. Extensive hospital course 4. Lung function could be normal or there could be obstructive or restrictive disease. Bronchial hyperactivity/dysfunction.

Bronchiolitis

Definition: acute inflammatory disease of lower respiratory tract that results in obstruction of small airways. Pathophysiology: 1. abnormal secretions combined with edema of submucosal layer cause obstruction of the small airways and diffusion impairment. Multiple areas of atelestasis produce V/Q mismatch and abnormal gas exchange. 2. hypoxemia, hypercarbia and respiratory failure develop when infant become fatigues and Ve fails. Etiology: 1. mostly RSV (60-85% of cases), 30% of cases infants are infected with multiple viruses. Other causes may include, rhinovirus, parainfluenza, adenovirus, and mycoplasma. RSV and adenovirus can cause long term complications. 2.Infants most at risk for RSV include those with major chronic pulmonary disease, CF, neuromuscular disorders, BPD, prematurity, immunodeficiencies, and CHD. More severe disease is seen in boys, infants who were not breastfed, and those of lower socioeconomic status. 3. most commonly occurs during winter and early spring. Clinical Presentation: 1. Hx: generally 3-7 day hx of URI with fever and known exposure. Usually lower grade temperatures. 2. Physical examination: cough, sneezing, rhinorrhea, RDS, increased WOB, prolonged expiration, rales, poor feeding. Tachypnea is most consistent clinical manifestation. possible crackles and wheezing. 3. Diagnostics: viral panel 4. Clinical Course: generally 7-10 days with peak around 48-72 hrs. severe cases may require mechanical ventilation. Patient Care Management: 1. most with mild signs can be treated outpatient. 2. nasal sxn/NP to improve breathing for infant to eat 3. NG feeding at times. 4. apnea may lead to mechanical ventilation 5. heated, humidified HFNC, flow delivers Positive pressure which keeps alveoli open to reduce V/Q mismatch 6. Hypertonic saline 3% should not be used in ER, but may be inpatient. 7. bronchodilators 8. steroids, chest physiotherapy and ABX are not recommended. Outcome: impaired oxygenation may continue for several weeks after apparent clinical recovery.

Tracheoesophageal fistula (TEF)

Definition: congenital anomaly in which the esophagus is segmented with a blind pouch separating the upper and lower portion, most of the time the fistula is attaching the esophagus to the trachea. Most common Type: esophageal atresia with distal tacheoesophageal fistula Type 2: isolated esophageal atresia H- Type: TEF without esophageal atresia Pathophysiology: interruption in the 4th week of fetal life (day 36) Clinical Presentation: 1. Maternal hx of polyhydramnios is common 2. presents with regurgitation of saliva, dx made by placing an NG into the pouch with an X-ray to confirm. 3. H-type presents at 3 to 4 months of age with hx of resp. distress, pneumonia, and cyanosis with feedings. 4. Bronch is diagnostic of choice. Patient Care Management 1. preoperative stabilization 2. may require vent support and NMB 3. OP or NP sxn that doesn't go past site of anastomosis. 4. pleural CT and drain 5. Gastric decompression (NG or GT), NG will stay in place to stent the site open. 6. transition from enteral feedings to PO. Outcomes: 1. Survival >95% 2. Most common complication is stricture or recurrent fistula formation 3. Persistent respiratory symptoms in 50% of cases (apnea, bradycardia, aspiration, recurrent pneumonia, and respiratory arrest) 4. largest cause of persistent respiratory disease is GERD, which is treated both medically and surgically.

Choanal Atresia

Definition: most common cause of true nasal obstruction. 1 in 10,000 births, can be unilateral or bilateral, isolated or associated with other congenital anomalies. Unilateral is 2x as common as bilateral. Pathophysiology: embryologic cause is unknown, theories point to failure of mesodermal flow to reach perordained positions in the facial process. Clinical Presentation: 1. clinical presentation my be severe with immediate respiratory distress that requires ETT or OA. 2. unilateral infants may be asymptomatic until unaffected nare is blocked with secretions. Bilateral infants classically appear normal when crying and mouth breathing, but respiratory difficulty appears as soon as they breathe through the nose. 3. can be diagnosed on CT, but MRI should be done to determine intracranial connections. Patient Care Management: 1. nasal causes of obstruction are relieved by an OA 2. surgical intervention includes opening the bony membrane that is blocking the airway and inserting tubes to maintain, these are sutured in for at least 4 weeks. Outcomes: 1. excellent and generally without complications. Most significant complication is restenosis, which is managed by repeated dilations. 2. rarely, long-term ETT or Trach is required when complicated by reconstructive maneuvers for other craniofacial abnormalities.

Bronchitis

Definition: nonspecific inflammation of the bronchioles, acute or chronic. Pathophysiology: 1. Acute: associated with viral illness, commonly, parainfluenza, RSV, and rhinovirus. inflammation leads to destruction of ciliated epithelium. A secondary infection can also develops due to weakened tissue 2. Chronic: symptoms last longer than 2 weeks and are usually a symptom of another condition. Clinical Presentation: 1. initial phase, upper respiratory symptoms 2. dry, brassy cough that may or may not be productive. 3. Coarse breath sounds of rhonchi Patient Care Management: 1. acute, care is supportive, including analgesia, hydration, antivirals, antibiotics, cough suppressants, and bronchodilators. 2. complications of pneumonia, rhinosinusitis or otitis can occur in chronically ill or undernourished children. 3. Treatment of chronic depends on the underlying cause. Outcome: malaise may continue for another week after the cough lessens.

Tracheal Stenosis

Definition: rare and potentially life-threatening defect that leads to severe respiratory insufficiency, most commonly complete vascular rings, severity related to length of trachea affected. Pathophysiology: 1. Narrowing of the tracheal lumen. Type A: Generalized hypoplasia (entire length is narrowed) Type B: Funnel-like Stenosis (subglottic tracheal diameter is normal, but distal narrows) Type C: Subsegmental stenosis (short segment of trachea is narrowed, hourglass fashion) 2. associated with abnormal bronchial branching patterns. 3. frequently associated with other cardiovascular and extrathoracic anomalies. Clinical Presentation: 1. Symptom dependent on severity of airway and degree of obstruction. 2. Minimal Symptoms, may be diagnosed incidentally or for a biphasic wheeze workup. Children/Adolescents might not be diagnosed until exercise associated respiratory difficulties. 3. Symptomatic Neonate, respiratory distress withing first few hours of life, presenting with coarse cough stridor, cyanotic spells. Assisted ventilation usually required. 4. Symptomatic Infant, presents with respiratory symptoms by end of 1st year with increase in physical activity, or increased respiratory illness. Symptoms of airflow limitation, wheeze, exertional shortness of breath, increased WOB. Patient Care Management: 1. some outgrow, but surgical intervention is often for symptomatic. 2. multidisciplinary treatment team 3. surgical options include rescection and primary anastomosis, rib cartilage tracheoplasty, pericardial patch tracheoplasty, and slide tracheoplasty. Slide Tracheoplasty is the preferred method. 4. no protocol for post-op period. 5. complications include anastomotic breakdown with subsequent air leak, tracheal narrowing r/t excessive granulation tissue or restenosis at the suture. Outcome: ENT follow up at discharge (Bronchoscopy and laryngosscopy)

Acute Laryngotracheobronchitis (LTB)

Definition: submucosa swelling in subglottic area. AKA "Croup" viral illness usually can be bacterial, usually preceded by upper respiratory illness. Pathophysiology: inflammation of subglottic region, trachea, bronchi, bronchioles with gradual onset. Parainfluenza viruses 1-3 75% of cases, other 25% is influenza A and B, adenovirus, and RSV. Secretions increase resistance to airflow leading to RDS. Clinical Presentation: usually in kids 3 months- 3yo, (peaks at 9 to 18 months) Hoarseness, barking cought, stridor, "steeple sign" on x-ray 1. Hx: recent URI, gradual onset, stridor at night 2. Physical Examination: Severity dependent on S/S. 3. Diagnostic Tests: CXR for the steeple sign 4. Clinical Course: monitor for cyanosis, pallor, weakness, or other signs of hypoxemia. Patient Care Management: Albuterol, steroids (dexamethasone), mist hydration, racemic epi, heliox for severe croup (lower density decreased turbulent flow of O2 and reduced WOB by decreasing the resistance), complication may include ETT Outcome: obstructive signs decrease over 3-4 days,

Neuromuscular blocking agents (NMB)

Description - depolarizing agents cause a continuous release and depletion of acetylcholine. - nondepolarizing agents bind the receptor sites of -acetylcholine so that synaptic transmission is blocked. Indications - ETT placement - control of ventilation with severe respiratory disease, increased ICP, or cardiac failure. - Reduction of VO2 (O2 consumption) from muscle movement NSG Considerations - paralytic is given with sedation and analgesia - ECG monitoring, Pox, - assess level of NMB agent regularly, peripheral nerve stimulation and drug holidays prolonged use associated with myopathy

Chloral Hydrate

Description: - alcohol based sedative-hypnotic - safe in children under 3 - not recommended in children over 4 or with neurodevelopmental disorders - CNS depressant, active metabolites trichloroethanol Indications: - use prior to nonpainful therapeutic or diagnostic procedures (EEG, MRI, CT, Ophtho exam, dental, infant PFT, ECHO) NSG Considerations: - monitor sedation level - VS - O2 sats prior and during procedure. tastes very bitter. Oral Dose: Infants and Children 25-50mg/kg/d divided Q6-8hr, max 500mg/dose Onset: 15-30 minutes Duration: 1-2 hr Contraindications: Hypersensitivity to Chloral hydrate or any component of the formulation, marked hepatic or renal impairment. Comments: Very limited use in US. Respiratory obstruction may occur in children with T&A hypertrophy, OSA, and Leigh's encephalopathy.

Ketamine

Description: - analgesic and amnestic medication - S receptor stimulation through SNS causes brief release of endogenous catecholamines. - increased HR, BP observed at low dose admin. - Dissocciative anesthesia in the eyes (nystagmus) Indications: - minimal effect on cardiopulmonary stability at doses less then 2 mg/kg. (good choice for unstable pts) - Bronchodilation = good for bronchospasm - increase ICP and cerebral blood flow NSG Considerations: - emergence phenomena observed in older children and adults. - always have bag/mask/ etc. emergency equipment, airway reflexes may be impaired. IV Dose: 1-2mg/kg over 30 to 60 sec; may administer additional doses of 0.5-1 mg/kg every 5-15 min PRN IM Dose: 4-5 mg/kg as a single dose; may give a repeate dose (2-5 mg/kg) if required Onset: <30 seconds Duration: 10-15 minutes Contraindications: hypersensitivity, conditions in which an increase in BP would be hazardous, infants younger than 3 months, known or suspected schizophrenia Comments: Analgesic, amnestic, hallucinogen, monitor for emergence delirium and nightmares, bronchodilator used for pts with asthma, prevent emergence reaction with bensodiazepines at end of procedure.

Benzodiazepines

Description: - cause anterograde amnesia through GABA inhibition in limbic system - little to no effect on retrograde memory - no analgesic properties - Increase in delirium with use. Indications: - short-term general sedation and amnesia for procedures - long-term for facilitating ventilation - acute management of seizures NSG Considerations: - use analgesic in conjunction with benzo - pt should be euvolemic (well hydrated) before administration to avoid hypotension. - prolonged use may result in dependence (sweating, anxiety, agitation, or hallucinations) - some pts exhibit a paradoxical response which worsens with increasing doses

Sildenafil

Description: - inhibits phosphodiesterase type 5 (PDE-5) in smooth muscle of pulmonary vasculature where PDE-5 is responsible for the degradation of cyclic guanosine monophosphate (cGMP). - increased cGMP results in pulmonary vasculature relaxation - vasodilation in pulmonary bed and the systemic circulation may occur Indication: - Pulmonary Hypertention (PH) NSG Considerations: - typically administered orally - pts avoid grapefruit juice it may increase serum levels - monitor HR, BP, and O2 - monitor for a prolonged erection in males, can lead to permanent disability.

Morphine and Morphine Like Opioids

Description: - opioid receptors are found in the brain and spinal cord - mu (M): supraspinal anesthesia; euphoria, respiratory depression, physical dependence - kappa (K): Spinal anesthesia; sedation, miosis, and respiratory depression - sigma (S): CNS stimulant; dysphoria, hallucinations, respiratory and vasomotor stimulation - most morphine like narcotics are M and K agonists. - nalbuphine K and S agonist and M antagonist, may reverse some M if administered after morphine Indications: - procedures - pain - continuous analgesia for mechanical ventilation - administer with a benzo for sedative effect. NSG Considerations: - vasodilatory effects from histamine release. - histamine release minimal with Fentanyl. - opioids induce central parasympathetic stimulation and depression of SA node (except meperidine) - dose-dependent respiratory depression - Fentanyl 100x analgesic potency or morphine, may cause chest wall rigidity with rapid/high dose administration

Surfactant Replacement Therapy

Description: - produced by type II alveolar epithelial cells in the lungs - composed mainly of lipids - surfactant helps prevent lung collapse during exhalation lessening the WOB optimizing surface area for gas exchange and V/Q matching, lung compliance, Indications: - prophylaxis and treatment of preterm infants with RDS - may be redosed in the fist 48 hours of presentation. - not used in pediatric acute respiratory disease (PARDS) - only in neonates NSG Considerations: - prophylactic admin. after stable in the first 15 min of life. - only administered via ETT - sedate and paralyze to avoid cough reflex - Side Effects: cyanosis, bradycardia, reflux into ETT - do not suction for 1hr following

Nitric Oxide

Description: - pulmonary vasodilator, relaxes pulmonary smooth muscle adjacent to functioning alveoli. - Atelectatic or fluid filled units will not uptake iNO. Indications - tx of hypoxic respiratory failure associated with clinical or ECHO evidence of Pulm. HTN in conjunction with ventilator support and other agents to improve oxygenation and reduce the need for ECMO. NSG Considerations - flow of 10-80ppm, doses of 20ppm should be used with caution. - extremely short half-life (~5 seconds) - once NO crosses vascular endothelium it is rapidly bound by Hgb forming methemoglobin the quantity of methemoglobin is dependent on iNO concentration. - NO metabolites are excreted by the kidneys as nitrates and nitrites. - Do not discontinue abruptively, rebound effects can occur.

Propofol

Description: - rapid onset with short duration - dose dependent effects - lipid like solution - bronchodilation and rapid recovery, ideal for deep sedation and induction. - global CNS depression through agonism of GABAA and reduced glutamatergic activity through NMDA receptor blockade. Indications: - in pediatrics use is limited to procedural sedation only. NSG Considerations: - may cause hypotension by direct vasodilation - egg and soy allergy pts can still receive, anaphylaxis occurs unrelated to allergies of soy or eggs. - prolonged infusions are not recommended. Dose: 0.5-2 mg/kg IV push, 50 microg/kg/min infusion Onset: 1-2 minutes Duration: 5-10 minutes Contraindications: hypersensitivity to drug or eggs, egg products, soybeans, or soy products. When general anesthesia or sedation is contraindicated. Comments: Helpful with refractory bronchospasm, caution with hypovolemia or CHF, may decrease ICP, may produce green urine (rare), does not provide analgesia.

Barbituates

Description: - sedation, not analgesic or amnestic agents - cardiopulmonary effects are dose dependent. - anticonvulsant properties - decrease cerebral metabolism (ICP) Indications: - short-term sedation for procedures and long-term sedation for ongoing management of assisted ventilation (rare) - increased ICP - continuous procedures with use of intra-aortic balloon pump (IABP). NSG Considerations: - higher doses, venodilation and depressed myocardial function and occur. - pulmonary effects are dose related, apnea occurs at mid dose range - alkaline solution, must be administered separate from other medications.

Dexmedetomidine

Description: - selective alpha2-adrenoceptor agonist with anesthetic and sedative properties due to activation of G-proteins by A2A receptors in brain stem resulting in inhibition of norepinephrine release. - peripheral A2A are activated at high doses or with rapid IV administration resulting in vasoconstriction. - pts continue to breathe, good choice for nonventilated pts. Indications: - initially intubated and mechanically ventilated - sedation prior to/and during surgery or other procedures of nonintubated pts - infusions should not exceed 24hrs NSG Considerations: - episodes of bradycardia, hypotension, and sinus arrest associated with rapid IV infusion - loading dose is optional - Transient hypertension: observed with loading dose administration, decreased infusion rate may be needed. - infusions longer than 24hrs associated with tolerance and tachyphylaxis and dose-related increase in adverse reactions. - prolonged use and abrupt discontinuation could lead to withdrawal symptoms, administer PO clonidine. - recovery times can be longer than propofol and ketamine use. - Assess level of sedation - VS (HR, RR, EKG, BP, and pain) Loading Dose: 0.5-1 mcg/kg over 10min Maintenance: 0.2-0.7 mcg/kg/hr (higher doses of 1 mcg are used) Onset: 30 minutes Duration: 4hrs Contraindications: none Comments: administer with infusion device set, loading dose over 10 minutes, may adhere to natural rubber.

Anti-inflammatory agents

Dexamethasone (Decadron) Methylprednisolone Description: - Asthma, ARDS, postextubation stridor use - will not provide immediate treatment for bronchospasm; effects are noted 24 to 36hrs after administration. Indications: - included in some daily asthma prevention plans - dexamethasone: treats upper airway edema edema and asthma exacerbations - methylprednisolone/prednisone: management and rescue in asthma exacerbations NSG Considerations: - Glucocorticoids interact with many other drugs - adverse reactions: NaCl & water retention, hyperglycemia, hypokalemia, HTN, and CNS changes ranging from dysphoria to mood disorders. - long-term therapy at risk for osteoporosis, ulcers, hyperlipidemia, increases susceptibility to infections. Leukotriene inhibitors (montelukast, Singulair) - block synthesis of c-leukotriene, a mediator that causes bronchoconstriction, mucus secretion, increased vascular permeability, and eosinophil migration to the airways - the release is not blocked by steroids - because of prolonged duration of onset not intended for acute episode of asthma Anticholinergic Agents (ipratropium, tiotropium) - used to treat bronchospasm with asthma and a bronchodilator in BPD and neonatal RDS. - block action of acetylcholine and parasympathetic sites in bronchial smooth muscle. - application to nasal mucosa inhibits serous and seromucous gland secretions

Remedial Agents: Pruritus (systemic opioids)

Diphenhydramine (Benadryl) 0.5-1 mg/kg IV Q6 Significant sedation may be observed

Hydomorphone

Dose Children <50kg: 0.015mg/mg/dose Dose Children >50kg: 0.2-0.6 mg/dose Onset: 15 minutes Peak Effect: 30-60 minutes Duration: 5hr Contraindications: Hypersensitivity, Acute or severe asthma, respiratory depression

Diazepam

Dose: 0.05-0.1 mg/kg Onset: 2-4 minutes Duration: 20-30 minutes Max. Dose: 0.6 mg/kg in an 8hr period Contraindications: Not recommended for continuous infusion, Severe hypotension, CNS depression Comments: prolonged effect because of long half-life and presence of active metabolites

Lorazapam (preprocedural in adults)

Dose: 0.05-0.1 mg/kg Onset: 3-5 minutes Duration: 20-30 minutes Contraindications: severe hypotension, CNS depression Comments: 4-12hr half-life may be used by intermittent bolus, no active metabolite

Pancuronium (IV)

Dose: 0.05-0.15 mg/kg Onset: 2-5 minutes Duration: 24 minutes Admin. over rapid injection Contraindications: Renal failure, 60-90% renal elimination Vagolytic: increased HR and BP

Vecuronium (IV)

Dose: 0.08-0.1 mg/kg Onset: 2.5-3 minutes Duration: 45-60 minutes Reconstitute to 1mg/1mL Contraindications: Liver Failure Renal elimination <25%

Atracurium (IV)

Dose: 0.3-0.5 mg/kg Onset: 2-3 minutes Duration: 20-70 minutes Undiluted bolus Contraindications: Hypotension, need for rapid onset Hoffman elimination makes it ideal for kidney or liver failure, very slow onset.

Rocuronium (IV)

Dose: 0.5-1.2 mg/kg Onset: 30-60 seconds Duration: 30-60 minutes Undiluted bolus Contraindications: none short duration

Remedial Agents: Respiratory Depression (benzodiazapines)

Flumazenil (Romazicon) <20kg: 0.01 mg/kg, repeat 0.005 mg/kg (max: 0.2 mg) 20-40kg: 0.2 mg (max: 1 mg) repeat 0.005mg/kg Caution in pts with seizure hx. Does not consistently reverse amnesia Used for reversal of conscious sedation May produce convulsions in patients physically dependent on benzos.

Midazolam

IV Dose: (Children 6 mo-5 yr): 0.05-0.1 mg/kg IV Dose: (Children 6 -12 yr): 0.025-0.05 mg/kg IV Dose: (Children 12 -16 yr): 0.02-0.04 mg/kg, titrate up to 0.1-0.2 mg/kg. IM Dose: (preprocedure): 0.1-0.15 mg/kg 30-60 min before procedure Onset: 2-4 minutes Duration: 20-30 minutes Max. Dose: 0.6 mg/kg Contraindications: Severe hypotension, CNS depression Comments: sedative-anxiolytic, anticonvulsant, alternate route of delivery including oral, rectal and transmucosal (nasal).

Methadone

IV Dose: 0.1mg/kg IM Dose: 0.1mg/kg/dose Q4hr for 2-3 doses then Q6-12 PRN Onset: 10-20 minutes Peak Effect: 1-2 hr Max. Dose: 10mg/dose Comments: Half-life 15-29hr, used for managing opioid dependence.

Succinylcholine (IV or IM)

IV Dose: 1-2 mg/kg IM Dose: 1-3 mg/kg Onset: 30-60 seconds Duration: 3-10 minutes Undiluted bolus, deep IM injection Contraindications: Myasthenia gravis, Malignant hyperthermia (MH), Avoid in pt with hyperkalemia, Guillain-Barre Syndrome, Skeletal muscle myopathies, crush, burn electrical injuries, open globe injury, increased ICP. Dysrhythmias Use a defasciculating dose Potassium efflux Muscle rigidity

Morphine

IV/IM Dose: 0.1-0.2 mg/kg (depending on age) Onset: pt dependent Oral (immediate release): ~30 minutes IV: 5-10 minutes Duration: immediate-release 3-5 hrs Considerations: Respiratory depression, metabolite accumulation in renal failure. Comments: May cause histamine release

Fentanyl

IV/IM Dose: 1-2 mcg/kg Onset: 3-5 minutes Duration: 0.5-1 hr Contraindications: severe respiratory depression Comments: Analgesic, mildly sedating

Remedial Agents: Nausea

Metoclopramide (Reglan) 0.1 mg/kg IV Q6h low incidence of side effects but extrapyramidal symptoms may occur Droperidol (Inapsine) 2-12 yr: 0.05 -0.06 mg/kg/dose Q4-6h > 12 yr: 2.5 -5 mg/dose Q4h can cause dysphoria, severe hypotension, extrapyramidal reaction Ondansetron (Zofran) 0.15 mg/kg IV x 3 doses Q4h no sedation; best used prior to anticipated stimulus (OR) low incidence of bronchospasm, seizures, headaches.

Remedial Agents: Respiratory Depression (Opioids)

Naloxone (Narcan) Mild Oversedation 0.01 mg/kg; repeat 2-3 min Opiate Intoxication <20 kg: 0.1 mg/kg >20 kg: 2 mg/dose Repeat 2-3 min until response noted May require repeat doses q20-60 minutes May cause abrupt cessation of pain control; sudden awakening with agitation, nausea and vomiting, and headache.

Remedial Agents: Pruritus (epidural/IV opioids)

Naloxone (Narcan) - opioid antagonist, displaced narcotics attached to receptors - used for complete or partial reversal Nalbuphine (Nubain) Continuous infusion 0.001 mg/kg/hr 0.01 mg/kg IV Q2-3 min This dose should not significantly reverse analgesia May improve analgesia May cause increased sedation

Medications that Affect V/Q Matching

Nitric Oxide Surfactant Replacement Therapy Sildenafil

Sputum or Aspirate Cultures

Normal: few to no segmented neutrophils (PMNs) PMNs: 3-4+ with dominant organism is more likely to be valid indicator of infection than one with <2+ PMNs and multiple organisms.

Remifentanil

Peak 20 min Duration: 4-5hr Contraindications: not for epidural or intrathecal administration. Comments: Monitor resp & CV status.

Apnuestic Breathing

Prologned, gasping inhalation followed by extremely short, ineffective exhalation; associated with brainstem insult. Severe Meningitis Extensive brain stem lesions

Sweat Chlorine Test

Sodium <70 Chloride <60 Potassium <60 higher levels suggest CF

Diagnostic Pulmonary Tests (PFTs)

Spirometry: can estimate lung volumes - obstructive disease pt cannot breathe out fully and has decreased Vc and FEV1 (forced expiratory volume). - restrictive disease lung cannot fully expand the Vc is low but FEV1 is normal. - weakened pt lower volumes with faster respiratory rate. Pressure Manometer: estimates muscle condition Measurement of Compliance: - lung compliance: (Cl) measure of distensibility of the lung and thoracic wall. volume of change per unit of pressure change across the lung (change V/change P) - Dynamic compliance: relationship of delivered volume to the total pressure required to deliver volume. includes elastic recoil and airway resistance. - Static compliance: at the end of a breath gas flow is paused. friction created by the flow disappears causing the inspirtory pressure to drop slightly. reflects the elastic properties of the lungs average distemsibility. (Normal range in infant and adult is 60-100 mL/cm H2O. Static compliance decreases with restrictive disease and increases with obstructive disease. Monitoring this helps determine Vt and optimal PEEP and prediction of weaning readiness. Obstructive Disease: decreased FEV, FVC (forced vital capacity), and FEV/FVC. Restrictive Disease: decreased FEV, FVC, and normal FEV/FVC. Asthma (restrictive and obstructive): decreased FEV, FVC, and normal FEV/FVC.

Differential WBC Count r/t pulmonary function

Total WBC <1 y: max=20,000 1-12 y: max=15,000 infections may cause elevated WBCs or very low (<4,000) Segmented Neutrophils (PMNs) <12 y = 25-40% > 12 y = >50% Band Neutrophils <10% increase in bands associated with bacterial infections Lymphocytes <12 y = >50% > 12 y = <40% increased with pertussis, Epstein-Barr Virus, Hepatitis Monocytes 4-6% Eosinophils 2-3% Basophils 0.5%

PVR equation

[80 x (mean pulmonary arterial pressure - mean pulmonary artery wedge pressure)] / Cardiac Output

Central neurogenic hyperventilation

a pattern of rapid and deep breathing caused by injury to the brain Midbrain/pontine infarcts Anoxia Midbrain Tumors Ischemia, low glucose to area > 24/min Increased VT No response to O2

PEP

administration of PEP from 5 to 20 cm H2O via facemask improves mucus clearance by increasing gas pressure behind secretions through ventilation or by preventing airway collapse during expiration.

Cheyne-Stokes

alternating periods of slow, irregular breathing and rapid, shallow breathing bilateral cerebral infarcts Encephalopathy due to HTN Metabolic diseases

cluster breathing

clusters of breaths follow each other with irregular pauses between Medullary tumor infarcts of medulla

Ventilatioin-Perfusion Scan (V/Q Scan)

injecting IV contrast and imaging its flow through the pulmonary vessels. comparison between areas of ventilation and perfused areas.

Ataxic breathing

irregular deep and shallow breaths cerebellar bleeds Pontine bleeding Severe Meningitis Compressing supratentorial tumors

FET (HUFF COUGH)

one or two forced expirations without closure of the glottis starting at middle to low lung volume, followed by relaxed breathing. Helps move secretions

Chest Physiotherapy

percussion, postural drainage, and vibration - helps make cough more effective - standard of care in pt with CF

Pulmonary angiography

peripheral catheter guided to the right side of the heart and to the pulmonary trunk. contrast in injected allows to definitive recognition of vascular obstruction.

Fluoroscopy

provides evaluation of thoracic motion Diaphragm movement in evaluation of paralyzed diaphragm

MRI

provides well defined visualization of soft tissue.

Oscillatory Devices (IPV)

therapy can be via mouth or chest wall (Vest) IPV: small busts of 200-300 cycles/min with aerosols assists in bronchodilation, from increased airway pressure, increased humidification, and cough stimulation.

ECHO

ultrasound waves used to detect structures and function of the heart and surrounding vasculature. noninvasive diagnostic tool to pulmonary hypertension, can be confirmed with a cardiac cath.

CT scan

used in identification of masses, fluid accumulation, and anatomic definition. Spiral CT may provide better definition and useful in eval. of pulmonary embolism (PE)


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