NPS Disease - OLD

Meconium Aspiration

• Meconium (fetal poo) can enter the respiratory system, clogging smaller airways. Ball-valve type obstructions (allows air in but no exhalation) can be particularly concerning
• Occurs mostly in near-term, term, and post-term scenarios
• Note clues: stained amniotic fluid is commonly given
• Avoid intubating, avoid initial intratracheal suction 
  Note: suctioning nose/mouth at birth is always indicated
• If bradycardia, fetal heart rate decelerations, or any sign of neo distress, be prepared to perform resuscitation (stimulate, bag-valve-mask if necessary)

Neonatal Pneumonia

• Increased risk if maternal infection (PROM with abdominal tenderness, fever, possibly contractions)
• Treat underlying infection with antibiotics 
• Use noninvasive to support distress if mild-to-moderate 
• If question suggests more severe, unresponsive (poor V/Q), consider supportive strategies to improve (don't just increase FIO2 but consider use of PEEP, patient positioning, HFOV)

Persistent Pulmonary Hypertension of the Newborn (PPHN)
Lungs persist in fetal circulation characteristic (high pulmonary pressure instead of transitioning to low pulmonary pressure)

• Nearly always term or post-term (preterm doesn't have developed vasculature!)
• Onset is at birth or very shortly after (within a few hours)
• Lungs stay as a high-pressure system instead of transitioning to a low pressure system (PVR remains elevated), causes right-to-left shunt
• To check: preductal/postductal SpO2:
  • Preductal: on right hand (always)
  • Postductal: on left hand or either leg/foot 
  • If preductal is higher by 10% or more, considered pulm hypertension: PPHN 
• Recommend echocardiogram (rule out congenital defects)

Treatment 

Calculate OI if possible, if not estimate it

1. Short trial of 100% O2 to reverse pulmonary vasoconstriction 
2. If mild symptoms, okay OI (< 25), use supplemental O2 
3. If mod-severe, OI > 25, recommend HFOV and/or iNO (or sildenafil)
4. If severe, OI > 25 (nearly always if > 40), recommend ECMO 

• If synchrony issue on vent (common): ensure pt trigger is set, consider NAVA, or use of sedation (opioid, etc.)
• Maintain adequate BP (may need inotropics, volume including PRBCs, milrinone if indication of ventricular dysfunction)
• Once improvement: wean oxygen/ventilator slowly. Expect "overreaction" to wean (small decrease in FIO2 or inspiratory pressure resulting in a sudden, dramatic desaturation with slow (hours) recovery). We manage this by making all weaning steps SLOW. 

Pediatric Pneumonia

• CXR: fluffy infiltrates are common, may be focused on one area or in more severe cases may be bilateral (approaching pediatric ARDS)
• Antibiotics if indication of bacterial, viral can consider antiviral if option (rare), fungal (very rare) would indicate need for antifungal 
• Treatment for pneumonia is largely supportive (if oxygen is failing, support it with positive pressure, maybe FIO2) and protective (lung protective tidal volumes) until enough time passes for underlying infection to clear.

Two pathways with Croup/Epiglottitis. 

If croup (think barking cough, steeple sign), supportive care to reduce symptoms is indicated. If epiglottitis (think no cough, drooling, thumb sign), an airway emergency is likely so don't delay care-get pt intubated safely. 

Croup

viral infection involving upper airway: laryngotracheaobronchitis, is the more common upper airway obstruction.  It occurs in infants/toddlers with gradual progression.  The child is likely to be hoarse with mild inspiratory and expiratory stridor.  A barking cough is very common.  Lateral neck x-ray may indicate presence of a "Steeple Sign," haziness in the subglottic area.

• Give racemic epinephrine and cool aerosol to reduce symptoms, closely monitor child 
• Steroids may be helpful (oral usually), Heliox may be helpful
• If mild (barking cough and possibly stridor during agitation): dose of steroids and monitor, likely discharged 
• If moderate (barking cough and stridor during rest, maybe increased WOB): steroids, nebulized epi 
• If severe (barking cough, stridor at rest, distress/increased WOB): steroids, nebulized epi, possibly intubate esp if change in LOC/lethargic

If intubating, expect to use a smaller ET tube than normal due to airway edema

Epiglottitis (supraglottitis)

life-threatening until airway secured

bacterial infection commonly caused by Haemophilus influenza B (other bacteria are possible), is a less common upper airway obstruction, but a much more serious one.  It appears in children over 3 yrs.  

• Symptoms: extreme anxiety, respiratory distress, stridor with drooling, tripod position, usually no cough. lateral neck radiograph may show "thumb sign" or haziness in supraglottic area.
• If any indication of epiglottitis in question, be cautious with interactions or risk airway loss
• If airway inadequate: try BVM ensuring chest rise, transport to OR if option. No chest rise? Intubate using fiberoptic equipment, smaller ET tube. Unable to intubate? Crich likely needed to establish airway.
• DO NOT recommend LMA (supraglottic airway device) in airway obstruction
• DO NOT delay with racemic epi neb (airway patency is critical)
• Typically responds within 72 hours to treatment which includes: maintain patent airway, antibiotics, IV steroids possibly. 
• Keep on ventilator until airway is patent again: check for ET tube leak, if there is a leak noted (either on graphics, auscultation, or measured in volume) go ahead and wean (SBT) and extubate
• Note tube size during cuff leak-if larger leak may not be present even if no edema

• Bronchopulmonary Dysplasia (BPD)-more commonly referred to as Chronic Lung Disease or Chronic Lung Disease of Prematurity for testing
• Key elements to remember: 
  • decreased compliance (difficulty ventilating)
  • airway obstruction (air trapping)
  • airway hyperreactivity (risk of infection, asthma)
  • pulmonary artery hypertension may be present
  • if pulm. hypertension, expect cor pulmonale (right heart failure)
• Pay attention to ABGs. If an abnormal baseline (compensated respiratory acidosis), make decisions in that context (treat pH, not PaCO2; treat O2 to acceptable, not normal)
• Consider additional supports: 
  • Difficulty ventilating, esp low compliance (consider furosemide to diurese lungs, increasing compliance some)
  • Difficulty weaning from vent (consider furosemide in addition to scheduled spontaneous breathing trials) 
  • Escalation of vent settings is common (increased PEEP, FIO2) during exacerbations/infections 
• If RSV with CLD, consider palivizumab prophylactically with RSV
• Avoid scheduled SABAs as maintenance, use for exacerbations
• Exacerbations:
  • Closely monitor fluid status (fluid retention can quickly affect lung status, may confirm this if question mentions JVD, pedal edema, pulmonary edema)
  • Use of antibiotics for any opportunistic infections 
  • Treat similar to asthma if signs of airway hyperreactivity

• A normal-appearing ABG with poor oxygenation, diminished breath sounds may indicate impending respiratory arrest-consider options that are aggressive (intubate)
• Peak Flow: concern if < 50% 
• Do not recommend antibiotics unless infection (fever, WBC)
• Drugs: SABAs (continuous if severe), steroids (IV if exacerbation in hospital), SAMAs are neutral (can give, but no strong evidence)
• Pay attention to how drug is given (neb vs. MDI vs DPI)
• Ventilator: I:E ratio is important (air trapping, so allow for adequate exhalation)
• Ensure adequate asthma education, including confirming proper inhaler usage, presence of asthma action plan (understand the basics of asthma action plan/colored zones)

Respiratory Distress Syndrome (RDS)

• Always related to prematurity. May be given young gestational age or low birth weight
• Presentation nearly always involves progressive respiratory distress usually within 2-3 hrs of birth: tachypnea/tachycardia, grunting, retractions, poor oxygenation on ABG (think P/F ratio), Chest radiograph will be diffuse bilateral infiltrates (the term ground-glass is often used), with or without air bronchograms
  • response to increased oxygenation will be less than normally expected (refractory hypoxemia)
  • may show signs of failed treatment if on basic oxygen 
• Keys to treatment:
  
  • Prevention: delay premature delivery if possible (tocolytics). If delivery is likely, give maternal steroids 1-2 days before delivery if possible 
  • Flow/pressure is more important than FIO2. Need to get lungs open (nasal CPAP, ventilator), moderate FIO2 is recommended
  • Ventilator: lung protection (avoid barotrauma, volutrauma, O2 toxicity), if failing CPAP, consider conventional ventilation. If failing conventional, consider HFOV or HFJV. 
  • Surfactant administration: consider within 2-hrs if inadequate response to CPAP 
  • Avoid fluid overload, maintain BP, maintain hematocrit, avoid overstimulation, consider caffeine citrate

Apneas

• May be indication of drugs (narcotics such as nalbuphine, opioids, etc.)
• Periodic Breathing: not connected to prematurity, more common after several weeks after birth, look for scenario of 5-10 sec apneas with no other symptoms. This is NORMAL, do not intervene
• Apnea of Prematurity: common but ABNORMAL in premature neonates 
  • Caffeine to stimulate neuro drive of breathing
  • Consider more serious if accompanied by desaturations (SpO2) and definitely if bradycardia (HR)-intervene by stimulating, bag if no response, start CPR if indicated
  • Higher risk of sudden death (SIDS) - recommend apnea monitoring at home, Car Seat Test 

Respiratory Syncytial Virus (RSV) affects the majority of children by the time they are 2-years-old.  Symptoms vary from those of a "common cold" up through life-threatening respiratory distress and needs.  Secretions are common as well as inflammation and bronchoconstriction. 

• Respiratory distress is common and can range in severity. One of the keys to understanding what to do next is in making a basic distinction between mild distress (mild retractions, minimal or no need for extra O2, slight tachypnea/tachycardia consistent with a possible fever) which requires no specific interventions, moderate distress (notable retractions, some need for O2), and severe distress (impending respiratory arrest, including marked retractions, hypoxemia, maybe extreme tachycardia and tachypnea). Choose SUPPORTIVE options to treat: 
  • antipyretics for fever
  • give oxygen if O2 is low (nasal cannula, etc.) 
  • if respiratory distress, try nasal CPAP, or may need ventilator.  
  • bronchodilators may help with bronchoconstriction (given anywhere from every 4-6 hours up to every hour!)
  • bronchial hygiene may help (suctioning the nose especially, but nasotracheal suction may be necessary)
  • Ribavirin is used only in very severe cases, and requires a special nebulizer setup (SPAG) 

• Pierré-Robin Syndrome: small mouth/chin, large tongue, U-cleft. May become cyanotic when supine, esp after feeding, recovers when position changed. Recommend prone, NPA if needed, trach if severe
  
  
• Down Syndrome: difficult airway due to large, flopping tongue
  
  
• Choanal Atresia: occlusion of the nasal cavity that is either unilateral or bilateral (usually symptoms if bilateral). Crying may relieve obstruction. Insert an oral airway, likely need for surgical intervention if bilateral. 
  
  
• Laryngomalacia: partial blockage of glottal opening due to softening of tissues. Results in inspiratory stridor, difficulty feeding, brief apneas. If severe (apneas with cyanosis), intubate, then recommend surgical intervention. 

• Avoid oxygen in cases where a shunt needs to be maintained (HLHS) 
• Oxygen may help close a shunt when needed, though this is rarely a correct answer (risks of O2 admin/toxicity are greater than the clinical benefits)
• Have a working knowledge of the major defects, including their abbreviations

Seizures

• Seizures may indicate something else is going on (adverse reaction to drug or treatment, neurologic condition, high fever, etc.). Treating the underlying cause, when there is one, is an appropriate step. 
• Ensure airway patency-intubate to protect airway (no LMA), decreases risk of aspiration. Keep intubated until seizures are controlled. 
• Control seizures with anticonvulsants (diazepam, clonazepam)

Brain Tumors

• Brain tumors may affect respiratory function depending on location (midbrain, etc.)-scenario will suggest this
• Can result in seizures, more difficult to treat (nonreversible usually, though may be controlled with drugs)

Hydrocephalus

• Larger than normal head size (may note increased head circumference) 
• Intracranial pressure will increase, may result in bulging fontanelle, seizures, vomiting
• Monitor ICP, consider CT, MRI, or Lumbar Puncture (LP)
• Treatment often involves shunt placement to drain fluid away from the brain
• Respiratory impacts might include varying respiratory pattern, periods of apnea, respiratory muscle weakness. Anticipate the need for airway clearance and potentially airway patency

Pediatric Airways

Upper Airway Obstructions

• Anticipate scenarios with difficult airways-use fiberoptic equipment when offered, ensure proper position of airway 
• Know the difference between a pediatric airway and an adult airway (glottis more anterior, narrowing of airway, etc.)
  
  

Tracheomalacia

• Most often due to longer-term artificial airway, especially if cuffed. 
• To prevent: ensure cuff pressure are kept reasonably low 
• Diagnosis includes bronch 
• Treatment temporarily might mean different sized tube (if trach, consider extra long distally), may need surgery 

Vocal Cord Paralysis 

• May affect one (unilateral) or both (bilateral) vocal cords
• Sometimes a complication of forceps of delivery, often a result of infection
  
  
• Unilateral Vocal Cord Paralysis (UVCP)
  • Diagnosis: flexible endoscope direct observation
  • Symptoms: weak voice, breathy cry, coughing/choking with feedings (causes aspiration which may lead to aspiration pneumonitis)
  • Treatment: observation, surgical 
    
    
• Bilateral Vocal Cord Paralysis (BVCP)
  • Diagnosis: flexible endoscope direct observation
  • Symptoms: feeding difficulty, inspiratory stridor, airway obstruction, voice/cry usually normal 
  • Recommend: swallow study, sleep study, cardiac ultrasound 
  • Treatment: Trach may be necessary until paralysis resolves, surgical widening of airway (with a goal of decannulating)

Vascular Ring

• Aorta or branches form a complete ring around the trachea and esophagus
• Symptoms: noisy or labored breathing, especially while eating, vomiting, dysphagia, persistent cough 
• Diagnosis: chest radiograph, CT, echo, bronch 
• Intervention: may need preoperative intubation (if respiratory distress). Small amounts of flexion/extension of the neck can cause repeated distress even after intubation 
• Treatment: surgical intervention, no long-term effects usually 
  Do not delay surgery if respiratory infection (surgery will facilitate clearing of infection) 

Immunocompromised

• Any suggestion of being immunocompromised (radiation/chemo, organ transplant, HIV, etc.) makes a person at increased risk of infection (we call these "opportunistic"), including RSV, influenzae, adenovirus, cytomegalovirus. Expect illnesses to be more frequent, severe
• Some infections, including pneumonia, may be caused by less common pathogens (if suspected recommend blood culture, respiratory culture)
• Efforts should be made to prevent infection including "reverse" or neutropenic isolation. Severe cases may be in a positive pressure room
• More likely to require broad-spectrum antibiotics (as gram-negative, the more serious type of infection is more likely). If no improvement, consider antifungals. Viruses such as RSV may benefit from antivirals (ribavirin in this case) 
• Some immune-compromised patients need prophylactic treatment for colonization. CF is a good example, where many patients are on TOBI to treat colonization and prevent infection even when no raging evidence of infection.

Shock

• Treat shock aggressively. Your best bet is to figure out the cause (is it anaphylactic? is it hypovolemic? etc.) and then treat it.
• Anaphylaxis: give epi 
• Hypovolemic: give fluids (blood product if bleeding) 
• Look for clues to indicate shock: sudden decrease in BP (maybe HR, SpO2) with symptoms to support the cause 

Trauma

• Pay close attention to clues. If bleeding consider blood product (PRBCs, platelets, etc.)
• Look for common trauma-related injuries: pneumothorax (esp tension pneumo!), lung contusions (often with outward bruising though not always), effusions (often symptomatic as effusion grows, maybe hemothorax)
• Expect WBC to go up initially (don't assume it is an infection; stress in general may increase WBCs). It is safe to say that infection is unlikely to be correct answer if question is noted to be within a day of a trauma. 
• Spinal Cord Injury: location of injury determines respiratory impairment. 
  C3 and above: ventilator, trach, phrenic nerve pacing 
  C3-C5: pay attention to clues, may have some impairment
  Below: anticipate need for airway clearance therapies 
• Drowning: resuscitation is critical (minimize hypoxic/anoxic period); intubation is likely, use PEEP for pulmonary edema and surfactant washout. If hypothermia, use heated humidity and warm IV fluids to slowly increase body temp. If severe hypothermia < 28C, ECMO. 

Heart Failure

• Heart failure, especially CHF, may occur in children, usually secondary to severe lung disease or a congenital heart defect. 
• For any suspicion of heart conditions, recommend an echocardiogram (noninvasive, can be done bedside) to further investigate
• Be careful of questions that suggest fluid challenges/resuscitation (may cause heart to overwork), consider diuretics as needed especially if symptoms (JVD, pedal edema)

Cystic Fibrosis

• Sweat chloride is common test (60 mmol is considered diagnostic), not effective usually in infants (consider DNA analysis, symptoms)
• Know symptoms including thick, tenacious secretions that are difficult to manage independently and cause a persistent, productive cough, foul-smelling stools (greasy, bulky), salty tasting skin, frequent lung infections, growth issues
• Airway clearance plan is going to be critical to many questions/scenarios 
• SABA = rescue 
• Dornase alfa = decreases viscosity of secretions
• TOBI, colimesthate = treat colonization (esp Pseudomonas aeruginosa)
• Lung transplantation if FEV, 6MWT, is poor 
• Acute Infection
  • Steroids 
  • Aggressive airway clearance 
• Pay attention to exhalation time, auto-PEEP on ventilator 

Neuromuscular

Spinal Muscular Atrophy

Muscular Dystrophy

Muscular Dystrophy

Genetic disorder with various types 

May result in respiratory muscle weakness-may need ventilator support, airway clearance, etc. (or may require minimal-to-no support) 

Sleep-Related

• Sleep apnea is more common in morbidly obese, and may be more pronounced in children when adenoid tonsils are prominent or when other anatomical differences (small chin, large tongue, etc.)
• A sleep study if symptoms, including daytime sleepiness, excessive snoring. A CPAP titration study is indicated initially and anytime setting seems inadequate (symptoms occur despite reported compliance with therapy).
• Treatment, very generally, should initiate with CPAP if indication of obstructions with desaturations. If CO2 retention evidence, NPPV (BiPAP) is indicated. 

Inhalation Injuries

• If an indication of smoke inhalation, put on 100% O2 (NRB, etc.) due to risk of carbon monoxide. SpO2 will be falsely high if CO is present. 
• If soot around outside of mouth or nose, intubate due to risk of swelling of airways (soot on mouth/nose suggests airways have been affected). 
• Recommend bronchoscopy after inhalation injury to a) diagnose severity of injury by physically viewing airways and then b) to help manage sloughing of cells (think of it like a sunburn that is bad enough to peel)