Tag Archives: pediatrics

PEM Playbook – Approach to Shock

Originally published at Pediatric Emergency Playbook on June 1,
2016 – Visit to listen to accompanying podcast. Reposted with permission.

Follow Dr. Tim Horeczko on twitter @EMTogether

Do we recognize shock early enough?

How do we prioritize our interventions?

Are we making our patient better or worse?

World wide, shock is a leading cause of morbidity and mortality in children, mostly for failure to recognize or to treat adequately.

So, what is shock?

Simply put, shock is the inadequate delivery of oxygen to your tissues.  That’s it.  Our main focus is on improving our patient’s perfusion.

Oxygen delivery to the tissues depends on cardiac output, hemoglobin concentration, the oxygen saturation of the hemoglobin you have, and the environmental partial pressure of oxygen.

At the bedside, we can measure some of these things, directly or indirectly.  Did you notice, however, that blood pressure is not part of the equation?  The reason for that is that blood pressure is really an indirect proxy for perfusion – it’s not necessary the ultimate goal.

The equation here is a formality:

DO2 = (cardiac output) x [(hemoglobin concentration) x SaO2 x 1.39] + (PaO2  x 0.003)

Shock CAN be associated with a low blood pressure,

but shock is not DEFINED by a low blood pressure.

Compensated Shock: tachycardia with poor perfusion.  A child compensates for low cardiac output with tachycardia and a increase in systemic vascular resistance.

Decompensated Shock: frank hypotension, an ominous, pre-arrest phenomenon.


Shock is multifactorial, but we need to identify a primary cause to prioritize interventions.

How they “COHDe”: Cardiogenic, Obstructive, Hypovolemic, and Distributive.

Cardiogenic Shock

All will present with tachycardia out of proportion to exam, and sometimes with unexplained belly pain, usually due to hepatic congestion.  The typical scenario in myocarditis is a precipitous decline after what seemed like a run-of-the-mill URI.

Cardiogenic shock in children can be from congenital heart disease or from acquired etiologies, such as myocarditis.  Children, like adults, present in cardiogenic shock in any four of the following combinations:warm, cold, wet, or dry.

“Warm and Dry”

A child with heart failure is “warm and dry” when he has heart failure signs (weight gain, mild hepatomegaly), but has enough forward flow that he has not developed pulmonary venous congestion.  A warm and dry presentation is typically early in the course, and presents with tachycardia only.

“Warm and Wet”

If he worsens, he becomes “warm and wet” with pulmonary congestion – you’ll hear crackles and seesome respiratory distress.  Infants with a “warm and wet” cardiac presentation sometimes show sacral edema – it is their dependent region, equivalent to peripheral edema as we see in adults with right-sided failure.

“Warm” patients – both warm and dry and warm and wet — typically have had a slower onset of their symptoms, and time to compensate partially. Cool patients are much sicker.

“Cold and Dry”

A patient with poor cardiac output; he is doing everything he can to compensate with increased peripheral vascular resistance, which will only worsen forward flow.  Children who have a “cold and dry” cardiac presentation may have oliguria, and are often very ill appearing, with altered mental status.

“Cold and Wet”

The sickest of the group, this patient is so clamped down peripherally that it is now hindering forward flow, causing acute congestion, and pulmonary venous back-up.  You will see cool, mottled extremities.

Cardiogenic Shock: Act

Use point-of-care cardiac ultrasound:

Good Squeeze? M-mode to measure fractional shortening of the myocardium or anterior mitral leaflet excursion.

Pericardial Effusion? Get ready to aspirate.

Ventricle Size? Collapsed, dilated, or normal.

Careful with fluids — patients in cardiogenic shock may need small aliquots, but go quickly to a pressor to support perfusion.

Pressor of choice: epinephrine, continuous IV infusion: 0.1 to 1 mcg/kg/minute.  The usual adult starting range will end up being 1 to 10 mcg/min.

Avoid norepinephrine, as it increases systemic vascular resistance, may affect afterload.

Just say no to dopamine: increased mortality when compared to epinephrine.


Obstructive Shock

Mostly one of two entities: pulmonary embolism or cardiac tamponade.

Pulmonary embolism in children is uncommon – when children have PE, there is almost always a reason for it – it just does not happen in normal, healthy children without risk factors.

Children with PE will either have a major thrombophilic comorbidity, or they are generously sized teenage girls on estrogen therapy.

Tamponade — can be infectious, rheumotologic, oncologic, or traumatic.  It’s seen easily enough on point of care ultrasound.  If there is non-traumatic tamponade physiology, get that spinal needle and get to aspirating.

Obstructive Shock: Act

Pulmonary embolism (PE) with overt shock: thrombolyse; otherwise controversial.  PE with symptoms: heparin.

Tamponade: if any sign of shock, pericardiocentesis, preferentially ultrasound-guided.


Hypovolemic Shock

The most common presentation of pediatric shock; look for decreased activity, decreased urine output, absence of tears, dry mucous membranes, sunken fontanelle.  May be due to obvious GI losses or simply poor intake.

Rapid reversal of hypovolemic shock: may need multiple sequential boluses of isotonic solutions. Use 10 mL/kg in neonates and young infants, and 20 mL/kg thereafter.

Hypovolemic Shock: Act

Tip: in infants, use pre-filled sterile flushes to push fluids quickly.  In older children, use a 3-way stop cock in line with your fluids and a 30 mL syringe to “pull” fluids, turn the stopcock, and “push them into the patient.

Titrate to signs of perfusion, such as an improvement in mental status, heart rate, capillary refill, and urine output.

When concerned about balancing between osmolality, acid-base status, and volume status, volume always wins.  Our kidneys are smarter than we are, but they need to be perfused first.


Distributive Shock

The most common cause of distributive shock is sepsis, followed by anaphylactic, toxicologic, adrenal,and neurogenic causes.  Septic shock is multifactorial, with hypovolemic, cardiogenic, and distributive components.

Children with sepsis come in two varieties: warm shock and cold shock.

Distributive Shock: Act

Warm shock is due to peripheral vascular dilation, and is best treated with norepinephrine.

Cold shock is due to a child’s extreme vasoconstriction in an attempt to compensate.  Cold shock is the most common presentation in pediatric septic shock, and is treated with epinephrine.

Early antibiotics are crucial, and culture everything that seems appropriate.


Shock: A Practical Approach

“How FAST you FILL the PUMP and SQUEEZE”


Sometimes things are not so cut-and-dried.  We’ll use a practical approach to diagnose and intervene simultaneously.

Look at 4 key players in shock: heart rate, volume status, contractility, and systemic vascular resistance.


First, we look at heart rate — how FAST?

Look at the heart rate – is it sinus?  Could this be a supraventricular tachycardia that does not allow for enough diastolic filling, leading to poor cardiac output?  If so, use 1 J/kg to synchronize cardiovert.  Conversely, is the heart rate too slow – even if the stroke volume is sufficient, if there is severe bradycardia, then cardiac output  — which is in liters/min – is decreased.  Chemically pace with atropine, 0.01 mg/kg up to 0.5 mg, or use transcutaneous pacing.

If the heart rate is what is causing the shock, address that first.

Next, we look at volume status.


Look to FILL the tank if necessary.  Does the patient appear volume depleted?  Try a standard bolus – if this improves his status, you are on the right track.

Now, we look at contractility.


Is there a problem with the PUMP?  That is, with contractility?  Is this in an infarction, an infection, a poisoning?  Look for signs of cardiac congestion on physical exam.  Put the probe on the patient’s chest, and look for effusion.  Look to see if there is mild, moderate, or severe decrease in cardiac contractility.  If this is cardiogenic shock – a problem with the pump itself.  Begin pressors.

And finally, we look to the peripheral vascular resistance.


Is there a problem with systemic vascular resistance – the SQUEEZE?


Look for signs of changes in temperature – is the patient flushed?  Is this an infectious etiology?  Are there neurogenic or anaphylactic concerns?  After assessing the heart rate, optimizing volume status, evaluating contractility, is the cause of the shock peripheral vasodilation?  If so, treat the cause – perhaps this is a distributive problem due to anaphylaxis.  Treat with epinephrine. The diagnosis of exclusion in trauma is neurogenic shock.  Perhaps this is warm shock; both are supported with norepinephrine.  All of these affect systemic vascular resistance – and the shock won’t be reversed until you optimize the peripheral squeeze.



The four take-home points in the approach to shock in children:

  1. To prioritize your interventions, remember how patients COHDe: Cardiogenic, Obstructive, Hypovolemic, and Distributive. Your patient’s shock may be multifactorial, but mentally prioritize what you think is the MAIN case of the shock, and deal with that first.
  2. To treat shock, remember: How FAST You FILL The PUMP and SQUEEZE: Look at the heart rate – how FAST.  Look at the volume status – the FILL.  Assess cardiac contractility – the PUMP, and evaluate the peripheral vascular tone – the SQUEEZE.
  3. In pediatric sepsis, the most common type is cold shock – use epinephrine (adrenaline) to get that heart to increase the cardiac output. In adolescents and adults, they more often present in warm shock, use norepinephrine (noradrenaline) for its peripheral squeeze to counteract this distributive type of shock.
  4. Rapid-fire word association:
  • Epinephrine for cardiogenic shock
  • Intervention for obstructive shock
  • Fluids for hypovolemic shock
  • Norepinephrine for distributive shock


Agha BS, Sturm JJ, Simon HK, Hirsh DA. Pulmonary embolism in the pediatric emergency department.Pediatrics. 2013 Oct;132(4):663-7.

Dellinger RP, Levy MM, Rhodes A, et al. Surviving sepsis campaign: international guidelines for management of severe sepsis and septic shock: 2012. Crit Care Med. 2013; 41:580-637.

Jaff MR et al. for the American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation; American Heart Association Council on Peripheral Vascular Disease; American Heart Association Council on Arteriosclerosis, Thrombosis and Vascular Biology. Management of massive and submassive pulmonary embolism, iliofemoral deep vein thrombosis, and chronic thromboembolic pulmonary hypertension: a scientific statement from the American Heart Association. Circulation. 2011; Apr 26;123(16):1788-830.

Levy B et al. Comparison of norepinephrine-dobutamine to epinephrine for hemodynamics, lactate metabolism, and organ function variables in cardiogenic shock. A prospective, randomized pilot study. Crit Care Med. 2011; 39:450.

Micek ST, McEvoy C, McKenzie M, Hampton N, Doherty JA, Kollef MH. Fluid balance and cardiac function in septic shock as predictors of hospital mortality. Crit Care. 2013; 17:R246.

Osman D, Ridel C, Ray P, et al. Cardiac filling pressures are not appropriate to predict hemodynamic response to volume challenge. Crit Care Med. 2007; 35:64-8.

Ventura AM, Shieh HH, Bousso A, Góes PF, de Cássia F O Fernandes I, de Souza DC, Paulo RL, Chagas F, Gilio AE. Double-Blind Prospective Randomized Controlled Trial of Dopamine Versus Epinephrine as First-Line Vasoactive Drugs in Pediatric Septic Shock. Crit Care Med. 2015;43(11):2292-302.

This post and podcast are dedicated to Natalie May, MBChB, MPHe, MCEM, FCEM for her collaborative spirit, expertise, and her super-charged support of #FOAMed.  You make a difference.  Thank you.



Undifferentiated Shock

Powered by #FOAMed — Tim Horeczko, MD, MSCR, FACEP, FAAP

Pediatric; Emergency Medicine; Pediatric Emergency Medicine; Podcast; Pediatric Podcast; Emergency Medicine Podcast; Horeczko; Harbor-UCLA; Presentation Skills; #FOAMed #FOAMped #MedEd

Croup: ED-focused Highlights

Authors: James Costakis, MD (EM Resident Physician, UW/Harborview, Seattle, WA), Siobhan Thomas-Smith, MD (Pediatrics Resident Physician, Seattle Children’s Hospital, Seattle, WA), and Rebekah Burns, MD (Pediatric Emergency Attending Physician, Seattle Children’s Hospital, Seattle, WA) // Edited by: Alex Koyfman, MD (@EMHighAK, EM Attending Physician, UTSW / Parkland Memorial Hospital) and Brit Long, MD (@long_brit)

Case 1

A 2-year-old boy presents with sudden onset of cough and difficulty breathing that woke him from sleep. Parents thought his breathing was labored and brought him to the ED. He has a history of reactive airway disease but has never been hospitalized.

Vitals in the ED:

Temperature 100F, heart rate 110, blood pressure 90/60, respiratory rate 30, oxygen saturation 98% on room air.

Exam is remarkable for a fatigued boy who refuses to speak. He has stridor at rest, tracheal tug, and moderate intercostal retractions. You do not appreciate wheezing on exam. He has a mild scattered erythematous rash on his chest and arms.


Case 2

A 10-year-old boy presents with fever and vomiting. Parents report two days of fever up to 104F, associated with myalgias, nausea, and mild cough. Vomiting was prominent today, and parents are worried that he is dehydrated. For the past day, his voice has become slightly hoarse, and his breathing audible.

Vitals in the ED:

Temperature 103F, heart rate 120, blood pressure 110/70, respiratory rate 26, oxygen saturation 91% on room air.

Exam is remarkable for suprasternal and supraclavicular retractions, as well as audible stridor at rest.


Physiology of Croup

Croup is a spectrum of illness characterized by varying degrees of inflammation in the upper respiratory tract, with possible involvement of the lower respiratory tract. Patients may have laryngotracheitis, laryngotracheobronchitis, or laryngotracheobronchopneumonitis. In 75% of cases, parainfluenza virus is responsible [6]. Otherwise, RSV, metapneumovirus, influenza, adenovirus, coronavirus, or even mycoplasma can cause a similar syndrome [6,8]. Some patients have recurrent bouts of upper airway edema causing a croup-like syndrome, which is referred to as spasmodic croup. This is thought to be potentially related to hypersensitivity to viral antigens.


Classic presentation

Croup primarily affects children from 6 months to 3 years of age, with a peak incidence of 5% per year in 2-year-olds [8]. Boys are 1.4 times more likely than girls to develop croup [8]. Typically, it occurs in the late fall or early winter [8]. Patients may or may not complain of a short prodromal upper respiratory infection. The acute phase of illness is characterized by stridor, barky cough, hoarseness, and sometimes fever. Symptoms often come on abruptly at night. Within 48 hours, most patients have recovered, but they may have lingering upper respiratory symptoms for about a week [4,9].


Diagnosis of Croup

Croup is diagnosed clinically. Chest X-ray and respiratory viral panel are sometimes used when one is considering an alternative diagnosis, but otherwise do not meaningfully affect the patient’s clinical course and are not recommended in uncomplicated croup [12].

The differential diagnosis of croup includes other causes of upper airway obstruction, gastroesophageal reflux, and allergic syndromes such as angioedema or spasmodic croup.

Other causes of upper airway obstruction include:

  • Bacterial tracheitis
  • Laryngomalacia
  • Tracheomalacia
  • Vascular rings
  • Epiglottitis (unlikely if vaccinated)
  • Foreign body aspiration
  • Peritonsillar abscess
  • Retropharyngeal abscess
  • Tracheo-esophageal fistula

A high index of suspicion for these alternative diagnoses is critical, particularly in patients who are presumptively diagnosed with croup but fail to follow the expected clinical course. Some red flags include:

  • Failure to respond to racemic epinephrine after 30 minutes
  • Trouble handling secretions
  • Oxygen requirement
  • Wheezing


How to Identify Sick Patients

Croup is common, accounting for 15% of ED visits by children with respiratory complaints and for 5% of ED admissions in children under 6 years of age [4,5]. Luckily, croup is usually a mild syndrome requiring minimal intervention – about 85% of children presenting to the ED have mild croup [10]. Only 1 to 3% of children with croup are intubated, and even then the mortality rate of children intubated for croup is only 0.5% [4]. Even so, early identification and aggressive treatment are critical in this subgroup of very sick children in order to maintain this low mortality rate.

How can we quickly identify patients who are in more severe respiratory distress? The Westley croup score has been around since the 1970’s and might help predict which patients need racemic epinephrine [3]. The score stratifies patients based on level of consciousness, cyanosis, stridor, air entry, and retractions. However, the score is typically used in research studies to quantify the efficacy of an intervention, and it has not been prospectively validated to predict mortality, intubation, or hospital admission.

At Seattle Children’s Hospital, a child is considered to have “severe” croup when they have stridor at rest, plus one of the following [17]:

  • Moderate intercostal retractions
  • Tachypnea
  • Agitation or restlessness
  • Fatigue
  • Difficulty speaking or feeding

These patients warrant racemic epinephrine, as discussed below. Notably, decreasing stridor can be an ominous sign, just as decreasing wheezing can suggest impending respiratory failure in asthmatics. Be on the lookout for lethargy, increasing fatigue, and worsening mental status. In the lethargic patient with decreasing stridor, decreased level of alertness, and hypoxemia, intubation may be required.



Historically, cool mist or humidified air was used to treat croup, but they are no longer recommended as studies have consistently failed to show clinical improvement with these interventions [13-15]. The primary adjuncts to support of airway, breathing, and circulation are dexamethasone and racemic epinephrine.

Dexamethasone has been found to improve the Westley score at 6 and 12 hours (but not at 24 hours), with a NNT of 5 to improve the score [2]. Patients typically require less epinephrine, spend less time in the ED or hospital (by about 12 hours), and have fewer return visits or readmissions (RR of return or readmission 0.5) when treated early with dexamethasone [2].

The dose of dexamethasone is 0.6 mg/kg, rounded to the nearest 2mg, up to a maximum dose of 16mg. Lower doses may be as effective, but some studies have seen more patients improved at 12 hours with the higher dose [16]. At Seattle Children’s Hospital, all children with croup of any severity receive dexamethasone. Repeat doses are rarely given [17].

Racemic epinephrine may help by causing mucosal vasoconstriction and decrease subglottic edema. It has been found to improve symptoms at 30 minutes, but the effect is normally gone by 2 hours [1].

The dose of racemic epinephrine is 0.5 mL of nebulized 2.25% solution, diluted in 3 mL of normal saline. At Seattle Children’s, this is given as soon as possible to children with “severe” croup, and can be re-dosed every 2 hours up to 3 times [17]. Further doses are typically given as an inpatient, and failure to improve after 3 doses suggests a possible alternative or concomitant diagnosis. This medication is most commonly given to those with stridor at rest.

There is conflicting data on whether heliox can be beneficial in croup. Most studies assessing heliox have looked at children with moderate to severe croup [18]. Heliox may improve the croup score, even compared to racemic epinephrine, starting at 90 minutes and lasting up to 4 hours, but no difference was found after 4 hours [18]. Overall, the data are conflicting, and at this point it is impossible to make a strong recommendation on administration of heliox. Currently, it may be considered as an adjunct therapy in a patient with severe croup with only partial response to racemic epinephrine.



Patients may have stridor with activity and still do well at home. However, stridor at rest warrants further intervention. Children should be able to talk and feed with minimal retractions. At Seattle Children’s, patients must be on room air and must not have received racemic epinephrine in the 2 hours prior to discharge [17].

Patients not meeting discharge criteria within 2 hours of dexamethasone are likely to require admission. Respiratory distress despite multiple doses of racemic epinephrine suggests likely need for ICU care and consideration of ENT consultation for direct laryngoscopy.


Case Resolution

Case 1

This patient may have classic laryngotracheitis from parainfluenza. The viral exanthem is non-specific. However, if you are concerned about anaphylaxis, it would not be wrong to administer intramuscular epinephrine. Otherwise, his stridor at rest and moderate intercostal retractions warrant racemic epinephrine in addition to dexamethasone.

Case 2

This patient is older than most patients with classic croup. Given his fever, age, and poor oxygenation, he requires consideration of a broad differential. Chest X-ray and viral panel are reasonable. He may have influenza causing a croup-like syndrome with stridor and respiratory distress, and may benefit from racemic epinephrine to decrease upper airway inflammation.


Pearls & Takeaways

  • Do not routinely obtain chest X-ray or respiratory viral panel in children with uncomplicated croup.
  • In patients who fail to respond to racemic epinephrine, or who are in significant respiratory distress, the differential must be initially very broad, with particular concern for bacterial tracheitis.
  • All patients with croup of any severity can benefit from dexamethasone.
  • Racemic epinephrine can help for a short time, and if it doesn’t, broaden your differential.


References / Further Reading

  1. Bjornson C, Russell K, Vandermeer B, Klassen TP, Johnson DW. Nebulized epinephrine for croup in children. Cochrane Database of Systematic Reviews 2013, Issue 10. Art. No.: CD006619.
  2. Russell KF, Liang Y, O’Gorman K, Johnson DW, Klassen TP. Glucocorticoids for croup. Cochrane Database of Systematic Reviews 2011, Issue 1. Art. No.: CD001955.
  3. Westley CR, Cotton EK, Brooks JG. Nebulized racemic epinephrine by IPPB for the treatment of croup: a double-blind study. Am J Dis Child. 1978 May;132(5):484-7.
  4. Johnson DW. Croup. BMJ Clin Evid. 2009; 2009: 0321.
  5. Cherry JD. Clinical practice. Croup. N Engl J Med. 2008;358(4):384–391.
  6. Rihkanen H, Rönkkö E, Nieminen T, et al. Respiratory viruses in laryngeal croup of young children [published correction appears in J Pediatr. 2008;153(1):151]. J Pediatr. 2008;152(5):661–665.
  7. Mazza D, Wilkinson F, Turner T, Harris C. Evidence based guideline for the management of croup. Aust Fam Physician. 2008 Jun;37(6 Spec No):14-20.
  8. Denny FW, Murphy TF, Clyde WA Jr, Collier AM, Henderson FW. Croup: an 11-year study in a pediatric practice. Pediatrics. 1983;71(6):871–876.
  9. Bjornson CL, Johnson DW. Croup. 2008;371(9609):329–339.
  10. Bjornson CL, Johnson DW. Croup-treatment update. Pediatr Emerg Care. 2005;21(12):863–870.
  11. Chan A, Langley J, Leblanc J. Interobserver variability of croup scoring in clinical practice. Paediatr Child Health. 2001;6(6):347–351.
  12. Swingler GH, Zwarenstein M. Chest radiograph in acute respiratory infections. Cochrane Database Syst Rev. 2008;(1):CD001268.
  13. Scolnik D, Coates AL, Stephens D, Da Silva Z, Lavine E, Schuh S. Controlled delivery of high vs low humidity vs mist therapy for croup in emergency departments. JAMA. 2006;295(11):1274–1280.
  14. Moore M, Little P. Humidified air inhalation for treating croup. Cochrane Database Syst Rev. 2010;(9):CD002870.
  15. Moore M, Little P. Humidified air inhalation for treating croup. Fam Pract. 2007;24(4):295–301.
  16. Kairys SW, Olmstead EM, O’Connor GT. Steroid treatment of laryngotracheitis: a meta-analysis of the evidence from randomized trials. Pediatrics. 1989;83(5):683–693.
  17. seattlechildrens.org/pdf/croup-pathway.pdf
  18. Moraa I, Sturman N, McGuire T, van Driel ML. Cochrane Database Syst Rev. 2013 Dec 7;(12):CD006822.

Pediatric Protective Custody

Author: Matthew Pirotte, MD (Assistant Professor, Assistant Residency Director, Department of Emergency Medicine, Feinberg School of Medicine) // Edited by: Alex Koyfman, MD (@EMHighAK, EM Attending Physician, UTSW / Parkland Memorial Hospital) and Brit Long, MD (@long_brit)

Consent for treatment has long been considered a cornerstone in ethical medical practice. Children are considered to lack capacity to consent under the law, and therefore consent for their treatment falls to their legal guardians. In 2011 the American Academic of Pediatrics issued a policy paper on emergency care for children which states that “a child’s legal guardian… is required to act in the best interests of the child. When a legal guardian refuses to consent to medical care or transport that is necessary to prevent death, disability, or serious harm to the child, law enforcement officers may intervene.”1 When an emergency physician (EP) is faced with a scenario in which he or she does not believe a legal guardian is acting in the best interests of the child, further investigation and possibly action is required. In cases where the child’s health or safety are in question, the EP may consider taking temporary protective custody of a minor under their care.

Case 1

A four-year-old female is sent to the emergency department (ED) by her pediatrician with her grandmother with the chief complaint of bruising. The child resides with the grandmother as a result of a care plan created by a prior contact with the Child Protective Services (CPS) during the child’s last visit to the emergency department for a similar complaint. During the EP assessment it becomes clear that the child has new bruising and bruising of various ages. A medical evaluation has been performed as an outpatient by the child’s primary care physician which excludes medical or hematologic etiologies of her diffuse bruising. During the last visit it was suspected that the child was being physically abused by her mother’s boyfriend and a requirement of the care plan was that she remain in the custody of her maternal grandmother with no access or visitation by the suspected abuser. Upon further questioning of the grandmother it becomes apparent that she does not believe that the mother’s boyfriend is abusing the child and instead is insistent that the bruises are the result of repeated falls from bed. CPS and Social Work (SW) are consulted but the grandmother continues to insist that the bruising is the result of falls rather than non-accidental trauma and refuses to acknowledge that the person under investigation is a potential abuser. She is evasive when asked if she can guarantee no further contact between the child and the mother’s boyfriend. The CPS worker is on scene but is considering discharge if the grandmother can promise no further contact. The EP elects to take protective custody of the child and admit her to the hospital until a more appropriate safety plan could be assembled.

Case 2

A seven-year-old female is brought to the emergency department by medics after a generalized tonic clonic seizure at school. The child has a long history of epilepsy managed by a pediatric neurologist with valproic acid. Review of the record also indicates that the child has difficult home situation; she is cared for by a working single mother who has two other children with frequent seizures. As part of a routine evaluation a valproic acid level is sent to the lab and returns at an undetectable level. The EP and social worker open a conversation with the mother about this undetectable level, and the mother states that she has been providing the child her medication on a regular basis with no missed doses. When the EP explains to the mother that this is not possible given that the child now has an undetectable blood level of valproic acid, the mother becomes angry and states that the EP and hospital laboratory are falsifying the value so as to remove the children from her home. The mother insists on immediate discharge and states she will continue to care for her daughter in the same manner she always has. The child’s neurologist is called and states the mother has frequent stressors and occasionally misses dose but that he is surprised by an undetectable level. Despite this he recommends discharge. Risk management is called and disagrees with the neurologist, stating that the child should not be allowed to leave the facility until a more complete evaluation in finished. Ultimately the EP makes the decision that this undetectable drug level is evidence of neglect and takes the child into protective custody.

The two cases described above both occurred at a quaternary care academic medical center in Illinois. The Illinois Abuse and Neglected Children Reporting Act states that “a physician treating a child may take or retain temporary protective custody of the child without the consent of the person responsible for the child’s welfare, if (1) he has reason to believe that the child cannot be cared for at home or in the custody of the person responsible for the child’s welfare without endangering the child’s health or safety; and (2) there is not time to apply for a court order.”

For most emergency care providers the decision to take protective custody of a child can be a difficult one.

In Case 1 the EP judged that the grandmother’s refusal to engage with the suspicion of the mother’s boyfriend as a potential source of harm to the child was a potential threat to the child’s safety. While there was no indication that the grandmother herself was perpetrating the abuse, the EP determined that no home care plan could adequately guarantee that the child would be safe.

In Case 2 the EP disagreed with a consulting physician about the safety of the child. The distinction between potential neglect and sub-optimal care and attention to a child can be a difficult one. According to the Child Abuse Prevention and Treatment Act, neglect is failing to provide for the child’s basic needs, including physical, educational, or emotional necessities. The definition encompasses a wide range, from allowing truancy or substance abuse to inadequate supervision or delaying health care. Other federal definitions limit physical neglect to situations that “seriously endanger the physical health of the child” and allow that “the assessment… requires consideration of cultural values and standards of care as well as recognition that [neglect] may be related to poverty.”2 If the child’s drug level had been sub-therapeutic but not undetectable or if the mother had admitted that her exhaustion from caring for 3 children led her to make medication errors would the EP have viewed the case in the same manner? In this case the mother’s angry denial of any lapses in medication administration was the key factor in the EP’s decision to take the child into protective custody.

Despite a general paucity of formal didactic training in child abuse, emergency medicine residents have been shown to be similar to pediatric residents in terms of their comfort with interpreting exam findings of abuse and discussion of said findings with authorities.3 However physicians in general under-report child abuse and may even be skeptical as to the benefits of reporting such cases to CPS.4,5 In a qualitative study, emergency care providers identified several barriers to optimal care in these challenging situations including time required to report suspected cases of abuse to CPS and potential negative consequences to providers such as protracted legal involvement.6 Some pediatric providers have even reported fear of personal retaliation from families.7 This is problematic and potentially criminal as physicians are mandated reporters in every state in the United States.

After the decision has been made to take protective custody, there are important considerations. Hospital security or local law enforcement should be consulted prior to disclosure to the accompanying guarding if one is present, as this has a high potential to be an emotionally charged conversation. The EP should remain objective in their discussion of facts with the guardian but clear and firm in their indication that the child leaving the facility with the guardian has been legally removed as an option. It is prudent to keep discussions with guardians focused on the safety of the child rather than specific accusations of mistreatment. Discussion of the case with the departmental administrator, hospital administrator, and risk management officer is encouraged. Children being removed from abusive or neglectful homes should have a careful physical examination and evaluation as they often have significant medical, nutritional, preventative, and psychological needs.8,9 The ability to carry out this examination at the level of comprehensive detail that should be undertaken will likely be outside the scope of practice of the EP, and consultation with a pediatrician or a clinician specializing in child mistreatment is strongly recommended. Careful documentation of objective findings and the physician’s thought process with respect to the potential of harm to the child is essential. Recommendations for documentation include specific concern for abuse or neglect, characteristics and location of all injuries, laboratory or radiographic abnormalities that support the diagnosis of abuse or neglect, severity of illness, and anticipated need for follow-up care.10

Key points

  • Emergency physicians are generally permitted to take temporary custody of children in particularly high risk situations where the safety of the child is in question.
  • Individual state laws may vary slightly, and it is important to be familiar with laws governing your sites of practice.
  • After a decision has been made to take temporary protective custody of a child, collaboration with hospital security, law enforcement, hospital administration, and child protective services is prudent.
  • Documentation should be detailed and include specific concerns about the safety of the child in question.
  • Many children taken into temporary protective custody by emergency physicians will have complex medical, psychological, and social needs. A multidisciplinary approach including consultation with a pediatric specialist is encouraged.


References/Further Reading:

  1. Committee on Pediatric Emergency M, Committee on B. Consent for emergency medical services for children and adolescents. Pediatrics. 2011;128(2):427-433.
  2. Isaacman DJ, Poirier MP, Baxter AL, Bechtel K, Pierce MC. Abuse or not abuse: that is the question. Pediatr Emerg Care. 2002;18(3):203-208.
  3. Starling SP, Heisler KW, Paulson JF, Youmans E. Child abuse training and knowledge: a national survey of emergency medicine, family medicine, and pediatric residents and program directors. Pediatrics. 2009;123(4):e595-602.
  4. Flaherty EG, Sege R. Barriers to physician identification and reporting of child abuse. Pediatric annals. 2005;34(5):349-356.
  5. Marshall WN, Locke C, Jr. Statewide survey of physician attitudes to controversies about child abuse. Child abuse & neglect. 1997;21(2):171-179.
  6. Tiyyagura G, Gawel M, Koziel JR, Asnes A, Bechtel K. Barriers and Facilitators to Detecting Child Abuse and Neglect in General Emergency Departments. Ann Emerg Med. 2015;66(5):447-454.
  7. Gunn VL, Hickson GB, Cooper WO. Factors affecting pediatricians’ reporting of suspected child maltreatment. Ambulatory pediatrics : the official journal of the Ambulatory Pediatric Association. 2005;5(2):96-101.
  8. Committee on Early Childhood A, Dependent C, American Academy of P. Health care of young children in foster care. Pediatrics. 2002;109(3):536-541.
  9. Simms MD, Dubowitz H, Szilagyi MA. Health care needs of children in the foster care system. Pediatrics. 2000;106(4 Suppl):909-918.
  10. Kellogg ND. Working with child protective services and law enforcement: what to expect. Pediatr Clin North Am. 2014;61(5):1037-1047.


Can’t Intubate Can’t Ventilate

Originally published at Pediatric EM Morsels on May 20, 2016. Reposted with permission.

Follow Dr. Sean M. Fox on twitter @PedEMMorsels


“Can’t Intubate Can’t Ventilate” is one of the frightening statements that causes massive surges of adrenaline in everyone. Unfortunately, most neural synapses don’t function well with that large surge of adrenaline, and it is, therefore, imperative to contemplate how to manage this scenario before it arises.  We have previously discussed Transtracheal Ventilation and have several videos to view, but let us review this important topic briefly once more. Can’t Intubate Can’t Ventilate: How Do I Oxygenate?


Can’t Intubate Can’t Ventilate: Anatomy Matters!

  • With larger children and adults, the can’t intubate can’t ventilate scenario often leads to the Cricothyrotomy.
  • In younger children and infants, the differences in anatomy make a traditional cricothyrotomy challenging.
  • In infants and young children:
    • Generous proportions of subcutaneous adipose tissue (chunky little babies are cute…) obscures landmarks.
    • The Hyoid bone is more prominent than the thyroid cartilage.
    • The Thyroid notch is often not palpable.
    • The Cricothyroid membrane is:
      • More horizontally positioned vs its typical vertical position
      • Small!
        • Around 8 years of age it is 1/2 the height and width of an adult’s
        • In neonates, the size is not sufficient enough to insert any commonly used rescue device. [Navsa, 2005]
  • The altered anatomy makes location of the cricothyroid membrane more difficult (if at all possible) and the small size may make it impossible to pass a large cric-tube through.


Can’t Intubate Can’t Ventilate: Go Transtracheal

  • Transtracheal ventilation has been used successfully in children as well as adults. [Frerk, 2015; Cote, 2009]
  • It may not “secure” an airway, but it will provide the patient with oxygen while you sort out the problem (and change your pants).
  • It is also easier than placing an IV in a child!
    • Locate the trachea!
      • If you are able to locate the cricothyroid membrane and it is large enough you can use it
      • Potential to use this catheter later to convert to a guidewire-assisted percutaneous cricothyrotomy. [Boccio, 2015]
    • Load a large gauge needle/catheter (14 gauge), ideally one that is reinforced(as simple peripheral IV catheters are prone to kink and become obstructed) onto a fluid-filled syringe.
    • Aspirate as you enter the skin at a 30-45 degree angle aimed caudally.
    • When you aspirate bubbles, you are in the airway! Advance the catheter and retract the needle.
    • Boom… done. High-Fives all around! {oh wait… we need oxygen!}


Can’t Intubate Can’t Ventilate: The Hard Part

  • The most difficult aspect of the procedure is not waiting too long to do it and leading to hypoxic insult.
  • The next most difficult aspect is figuring out how to connect oxygen to the tiny catheter you just placed in the neck.
  • This is where contemplation of how to do this before you need to do it is important, because most of us are not going to successfully “MacGyver it” on the fly.
  • Oxygen Connection Options

    1. Commercial products
      • Have flow regulators that are easy to use. [Cote, 2009]
      • Connect easily via Lure-lock to the catheter.
      • Many have pressure regulators as well.
      • Con = Expensive.
    2. Oxygen Tubing and High Flow O2 from Wall 
      • Not as optimal as commercial products, but may be best you have available.
      • Turn flow up all of the way. [Bould, 2008]
      • Need to “MacGyver” a flow regulator and a connector
        • Flow Regulator
          • Cut large holes (several) in side of oxygen tubing.
          • Need large/multiple holes to allow air flow to egress easily and not add to PEEP. [Sasano, 2014]
          • May also use Y-connector to another oxygen tube.
        • Connector
          • 3-way stop cock can be used to fit into distal end of oxygen tubing and Lure-lock onto the catheter.
          • Need to ensure 3 way valve is open to flow!
    3. Self-Inflating Ventilation Bag [Sasano, 2014]
      • Not as optimal as commercial products, but may be best you have available.
      • 3.0 ETT bag connector
        • Remove from ETT
        • Insert distal end into catheter
      • 7.5 ETT bag connector
        • Remove from ETT
        • Insert into proximal end of 3 mL syringe (after removing the plunger).
        • Use Lure-lock on syringe to connect to catheter
      • Will need to disengage the bag’s pop-off valve.
  • Oxygenate!
    • Occluding the flow regulator will lead to airflow into the trachea (inspiration).
    • Uncovering the flow regulator will allow air flow from oxygen source and patient to escape (expiration).
    • Inspiration : Expiration = 1 second :  4 seconds
    • Use longer expiration phases for completely occluded upper airway (ex, 1:9)
      • Patient will tolerate hypercapnia better than barotrauma/pneumothorax.


Moral of the Morsel

  • Do not let the first time you think about transtracheal ventilation be when you realize you need to do it.
  • Know what equipment you have available.
    • If you have a commercial product, know how to use it and where it is.
    • If you don’t have a commercial product, make your MacGyver survival bag and keep it handy with the tools you need, so you don’t need to recall how to do it in the time of need.



Boccio E1, Gujral R2, Cassara M3, Amato T4, Wie B5, Ward MF6, D’Amore J7. Combining transtracheal catheter oxygenation and needle-based Seldinger cricothyrotomy into a single, sequential procedure. Am J Emerg Med. 2015 May;33(5):708-12. PMID: 25791154. [PubMed] [Read by QxMD]

Frerk C1, Mitchell VS2, McNarry AF3, Mendonca C4, Bhagrath R5, Patel A6, O’Sullivan EP7, Woodall NM8, Ahmad I9; Difficult Airway Society intubation guidelines working group. Difficult Airway Society 2015 guidelines for management of unanticipated difficult intubation in adults. Br J Anaesth. 2015 Dec;115(6):827-48. PMID: 26556848. [PubMed] [Read by QxMD]

Bould MD1, Bearfield P. Techniques for emergency ventilation through a needle cricothyroidotomy. Anaesthesia. 2008 May;63(5):535-9. PMID: 18412654. [PubMed][Read by QxMD]

PEM Playbook – Altered Mental Status in Children

Originally published at Pediatric Emergency Playbook on May 1,
2016 – Visit to listen to accompanying podcast. Reposted with permission.

Follow Dr. Tim Horeczko on twitter @EMTogether


How do you approach the child who may be altered?

Altered mental status in children can be subtle.  Look for age-specific behaviors that range from irritability to anger to sleepiness to decreased interaction.

In the altered child, anchoring bias is your biggest enemy.  Keep your mind open to the possibilities, and be ready to change it, when new information becomes available.

For altered adults, use AEIOU TIPS (Alcohol-Epilepsy-Insulin-Overdose-Uremia-Trauma-Infection-Psychosis-Stroke).

Try this for altered children: remember that they need their VITAMINS!

V – Vascular (e.g. arteriovenous malformation, systemic vasculitis)

I – Infection (e.g. meningoencephalitis, overwhelming alternate source of sepsis)

T – Toxins (e.g. environmental, medications, contaminated breast milk)

A – Accident/abuse (e.g. non-accidental trauma, sequelae of previous trauma)

M – Metabolic (e.g. hypoglycemia, DKA, thyroid disorders)

I – Intussusception (e.g. the somnolent variant of intussusception, with lethargy)

N – Neoplasm (e.g. sludge phenomenon, secondary sepsis, hypoglycemia from supply-demand mismatch)

S – Seizure (e.g. seizure and its variable presentation, especially subclinical status epilepticus)

Case One: Sleepy Toddler

16-month-old who chewed on his grandmother’s clonidine patch

Clonidine is an alpha-2 agonist with many therapeutic indications including hypertension, alcohol withdrawal, smoking cessation, perimenopausal symptoms.  In children specifically, clonidine is prescribed for attention deficit hyperactivity disorder, spasticity due to cerebral palsy and other neurologic disorders, and Tourette’s syndrome.

The classic clonidine toxidrome is altered mental status, miosis, hypotension, bradycardia, and bradypnea.  Clonidine is on the infamous list of “one pill can kill”.

Treatment is primarily supportive, with careful serial examinations of the airway, and strict hemodynamic monitoring.

Naloxone can partially counteract the endogenous opioids that are released with clonidine’s pharmacodynamics.

Start with the usual naloxone dose of 0.01 mg/kg, up to the typical adult starting dose of 0.4 mg.

In clonidine overdose, however, you may need to increase the naloxone dose (incomplete and variable activity) up to 0.1 mg/kg.  Titrate to hemodynamic stability and spontaneous respirations, not full reversal of all CNS effects.

Case Two: In Bed All Day

A 7-year-old with fever, vomiting, body aches, sick contacts.  Altered on exam.

Should you get a CT before LP?

If you were going to perform CT regardless, then do it.

Adult guidelines: age over 60, immunocompromised state, history of central nervous system disease, seizure within one week before presentation, abnormal level of consciousness, an inability to answer two consecutive questions correctly or to follow two consecutive commands, gaze palsy, abnormal visual fields, facial palsy, arm drift, leg drift, and abnormal language.

Children: if altered, and your differential diagnosis is broad (especially if you may suspect tumor, bleed, obvious abscess).

Influenza is often overlooked as a potential cause of altered mental status.  Many authors report a broad array of neurological manifestations associated with influenza, such as altered mental status, seizures, cranial nerve abnormalities, hallucinations, abnormal behavior, and persistent irritability.  All of this is due to a hypercytokinemic state, not a primary CNS infection.

Case Three: “Terrible Teenager”

14-year-old brought in for “not listening” and “acting crazy”; non-complaint with medications for systemic lupus erythematosus (SLE).

SLE is rare in children under 5. When school-age children present with SLE, they typically have more systemic signs and symptoms.  Teenagers present like adults.  All young people have a larger disease burden with lupus, since they have many more years to develop complications.

Lupus cerebritis: high-dose corticosteroids, and possibly IV immunoglobulin.  Many will need therapeutic plasma exchange (TPE), a type of plasmapheresis, where the patient’s plasma is replaced with donor plasma, to remove auto-antibodies and cytokines.


  • In altered mental status, keep your differential diagnosis open
  • Pursue multiple possibilities until you are able to discard them
  • Be ready to change your mind completely with new information
  • Make sure your altered child gets his VITAMINS (Vascular, Infectious, Toxins, Accident/Abuse, Metabolic, Intussusception, Neoplasm, Stroke)


Beckman HB, Frankel RM. The effect of physician behavior on the collection of data. Ann Intern Med 1984; 101:692.

Fujita K, Nagase H, Nakagawa T et al. Non-convulsive seizures in children with infection-related altered mental status. Pediatrics International. 2015; 57(4):659–664.

Gallagher J, Luck RP, Del Vecchio M. Altered mental status – a state of confusion. Paediatr Child Health. 2010 May-Jun; 15(5): 263–265.

Hasbun R, Abrahams J, Jekel J, Quagliarello VJ. Computed tomography of the head before lumbar puncture in adults with suspected meningitis. N Engl J Med. 2001; 345(24):1727-33.

Oliver WJ, Shope TC, Kuhns LR. Fatal Lumbar Puncture: Fact Versus Fiction—An Approach to a Clinical Dilemma. Pediatrics. 2003; 112(3)

Schwartz J et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice—Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Sixth Special Issue. Journal of Clinical Apheresis. 2013; 28:145–284.

Zorc JJ. A lethargic infant: Ingestion or deception? Pediatr Ann 2000; 29: 104–107

This post and podcast are dedicated to Teresa Chan, HBSc, BEd, MD, MS, FRCPC for her boundless passion for and support of #FOAMed, for her innovation in education, and for her dedication to making you and me better clinicians and educators.  Thank you, T-Chan.



Altered Mental Status

Powered by #FOAMed — Tim Horeczko, MD, MSCR, FACEP, FAAP

Ear, Nose, and Throat Foreign Bodies

Authors: Brooke Moungey, MD (Senior EM Resident at the University of Wisconsin) and Christopher Stahmer, MD (Clinical Assistant Professor at the University of Wisconsin) // Edited by: Jennifer Robertson, MD, MSEd and Alex Koyfman, MD (@EMHighAK, EM Attending Physician, UTSW Medical Center / Parkland Memorial Hospital)

It is a busy day in the emergency department (ED) when a 35-year old male presents with an insect in his right ear. In the next room, a 3-year old girl presents with her mother after having placed a plastic bead in her left nostril. And just then, a 2-year-old boy is brought in to the ED by his parents because he had just swallowed a coin.

With all the possible tools and techniques for foreign body removal, choosing a method that is most likely to be successful for your patient can sometimes be a daunting task. In addition, it can be challenging when trying to determine if a patient will be cooperative and whether analgesia or sedation will be required.

Here are a few tips to help you remove those foreign bodies like a pro and get your patient safely on his or her way.

Foreign Bodies of the Ear Canal:

While foreign bodies in children often include a wide range of objects such as toys, rocks, beads, crayons, and cotton swabs, foreign bodies in adults are most commonly insects.

There is a wide array of tools available to assist in removal and this can often be confusing for providers as these tools are not created equal for all types or locations of foreign bodies.

  • You should always check for tympanic membrane (TM) perforation. If this is present, the patient should not undergo removal in the ED and should be referred to otolaryngology (ENT) for removal.
  • In general, you should avoid sharp instruments if you are not getting adequate visualization of the foreign body. Additionally, sharp objects should not be used if the patient is not cooperative with your exam so that accidental TM damage can be avoided.
  • Side effects of these removal techniques include tissue damage such as abrasions, lacerations or TM perforations. These techniques can also push the object further back in the ear canal and make removal even more difficult.
  • There are several different types of otoscopes. Some include those with removable lenses and operating otoscopes that allow tools to pass through the otoscope head while directly visualizing foreign bodies.


Alligator forceps and curettes:

Curettes are best for foreign bodies that can be well visualized but are small enough that the curette can be pushed beyond the object and pulled from behind. Forceps can also be used to directly grasp objects but are less effective when used for round objects or larger objects.  They are more effective for soft or irregularly shaped objects that can be grasped firmly to prevent pushing them further out of reach.



Can be done using a 20 mL syringe and 16 gauge angiocatheter filled with water or normal saline. A plastic basin may be used to catch the fluid. This technique can be helpful for a variety of foreign body shapes and sizes and is safe for normal TMs. However, irrigation should not be used in patients with a history of TM perforation or instrumentation including myringotomy tubes. This method should not be used for foreign bodies made of organic material as they will soak up the fluid and expand. This will cause further difficulty with extraction. Irrigation should also never be used if the foreign body is a button battery.



Suction tpically requires 100 mmHg or more of negative pressure with a soft-tipped plastic suction catheter placed against the object. The side port can be occluded to capture the object and pull it out. Tip: Best for hard, round foreign bodies.



Can be placed on the end of a cotton-tipped applicator that is advanced into the ear canal and pressed against the foreign body until dry. The foreign body can then be pulled out.  Be very cautious as glue can be accidentally applied to the ear canal or TM itself and cause tissue damage.

Tip: Best used to extract round objects which are well visualized.



Can be used for removing metal foreign bodies. It works best for smaller metal objects and those in the more distal portion of the ear canal.


Can be manually extracted with forceps or a curette, or irrigated out of the ear canal as previously described.   However, live bugs often cause significant patient discomfort with attempted removal. This can be prevented by using a syringe and angiocatheter to fill the ear canal with viscous lidocaine and allowing it to sit for 15 minutes prior to removal.  Mineral oil is also an alternative to viscous lidocaine.


A small amount of viscous lidocaine can be a helpful local anesthetic. However, sedation may be needed in younger pediatric patients. Several common options include:

  • Intranasal midazolam 0.3-0.5 mg/kg
  • Oral midazolam 0.5 mg/kg
  • Ketamine IV 1 mg/kg or IM 2-3 mg/kg

Take Away Points:

  • Referral to ENT will be necessary if extraction in the ED fails, the foreign body has been in place long enough to cause acute otitis media or other surrounding infection, or if there is a TM perforation.
  • Antibiotics will be necessary for abrasions or lacerations of the ear canal, even if extraction is successful. Prescribe ofloxacin 0.3% otic drops 10 drops once daily for 7 days.
  • Use different tools and techniques to your advantage to successfully address different types of foreign bodies in the ear canal.
  • When one foreign body is found, check for additional foreign bodies in the ears, nose and mouth (especially in pediatric patients).
  • Never apply a liquid to a button battery.

Nasal foreign bodies:

Nasal foreign bodies are most commonly found in pediatric patients and often include many of the same objects as foreign bodies in the ear canal. Once the foreign body is located, there are several easy techniques to remove them without additional instrumentation.

  • Side effects of these removal techniques include abrasions, lacerations, and epistaxis. Another side effect is potentially moving the object further back, which will increase the risk of aspiration or ingestion.
  • A nasal speculum is beneficial to aid in visualizing the object.


Positive pressure:

If the patient can assist with removal, then nose blowing while sitting forward and occluding the other nostril is often effective.  If the child is unable to participate in nose-blowing the “big kiss” technique is an alternative. With this technique, have the parent blow into the child’s mouth while occluding the patent nostril.  This can also be modified to apply high flow supplemental oxygen or a Bag Mask Valve (BVM) into the mouth while again occluding the patent nostril.  Please use caution due to a risk of barotrauma if oxygen or a BVM is used.


Balloon catheters:

Small balloon catheters such as a Fogarty or Katz extractor can be attached to a 5 mL syringe. Check the balloon function and then advance the deflated catheter tip into the nostril until the entire balloon is past the foreign body. Then inflate the balloon until it fills the space behind the object and pull the balloon catheter out along with the object.



Can also be used similarly to foreign bodies of the ear canal. However, they are more effective for nasal foreign bodies if advanced past the object and then used to pull the object forward. If they are used to directly grasp the object, then the object can be pushed further back, which may lead to aspiration, ingestion, or more a difficult extraction.


Lidocaine or phenylephrine spray can be used to prevent swelling and pain, but should be rarely used due to the risk of causing sneezing and associated aspiration or ingestion of the object.  Both of these medications should be avoided in the case of button batteries.

Sedation options are similar to those as listed above for ear foreign bodies; however, no intranasal medications such as intranasal midazolam should be used due to risk of aspirating/ingesting the object.

Take Away Points:

  • Referral to ENT will be necessary if ED extraction fails, extraction causes uncontrolled epistaxis or if the foreign body is aspirated.
  • When possible, avoid anesthetic sprays and intranasal medications to prevent aspiration or ingestion.
  • Non-invasive techniques with positive pressure and the assistance of the child and/or parents are often most successful for nasal foreign bodies.
  • Always look for other foreign bodies and never apply liquids to a button battery.

Foreign Bodies of the Throat:

In adults, these foreign bodies often represent food boluses, fish bones, and dentures. In children, they may include a number of objects from coins and button batteries to food, toys, and crayons. If a patient presents with stridor or respiratory distress, it is best to leave him or her in a position of comfort while getting airway equipment ready and gathering resources such as anesthesia and ENT, if available. Airway intervention may become necessary at any time and can include attempting to remove the foreign body with Magill forceps if visible or intubating the patient with an attempt to force the object into the right mainstem bronchus to relieve a complete obstruction.


For patients who are not in respiratory distress, radio-opaque foreign bodies can be located in the esophagus or trachea/bronchus with AP and lateral neck X-rays and an abdominal x-ray can often be used to identify objects in the stomach or further along the GI tract. If the object is radiolucent and suspected to have been aspirated due to respiratory symptoms, then bilateral decubitus X-rays may show air trapping in the affected side such that the lung does not deflate appropriately when the affected side is down. Inspiratory and expiratory films may also show air trapping on the affected side during expiration and pushing of the mediastinum to the opposite side. Any objects located in the respiratory tract will likely have to be removed by bronchoscopy.

Esophageal Foreign Bodies:

Objects that are blunt and small (<2.5 cm wide) may be pushed forward into the stomach as they will likely be small enough to pass the pylorus and pass from the GI tract on their own. This is typically done with application of lidocaine spray to the oropharynx and advancement of a bougie which the patient swallows and is then advanced until the object is dislodged. If this is not an option or this method fails then endoscopy will be needed for removal. Any objects that are sharp, regardless of their location in the GI tract, or too large to pass the pylorus must also be removed by endoscopy. If there are signs the object has caused a perforation, then surgery will likely be indicated for definitive care.

Button batteries:

Regardless of whether the button battery is located in the respiratory tract or GI tract, this is a medical emergency and the battery should be removed by endoscopy or bronchoscopy within 6 hrs of aspiration to prevent necrosis or perforation.

Food bolus:

Administration of glucagon is unlikely to be of benefit as its use in clearing a food bolus is likely related to causing retching and emesis, which can also increase the risk of perforation or tearing of the esophageal mucosa. Food boluses can also be pushed forward into the stomach or removed by endoscopy as listed above for blunt objects in the esophagus.

Take Home Points:

  • Most foreign bodies of the throat will be aspirated or ingested and will likely require bronchoscopy, endoscopy or surgery for appropriate treatment. Thus, get your specialty services on board early.
  • Button batteries as a foreign body in any location are an emergency and every effort should be made to remove them as quickly as possible, preferably within 6 hrs of ingestion.
  • Small blunt foreign bodies (<2.5 cm) and food boluses can be pushed forward into the stomach and passed safely.
  • Always evaluate your patient for signs of perforation even after the foreign body has been removed.

References / Further Reading

-Baker MD. Foreign bodies of the ears and nose in childhood. Pediatr Emerg Care 3: 67, 1987.

-Botma M, Bader R, Kubba H. “A parent’s kiss”: evaluating an unusual method for removing nasal foreign bodies in children. J Laryngol Otol 114: 598, 2000.

-Figueiredo RR, Azevedo AA, Kos AO, Tomita S. Complications of ENT foreign bodies: a retrospective study. Rev Bras Otorrinolaringol (English ed) 74: 7, 2008.

-Kadish HA, Corneli HM. Removal of nasal foreign bodies in the pediatric population. Am J Emerg Med 15: 54, 1997.

-Kalan A, Tariq M. Foreign bodies in the nasal cavities: a comprehensive review of the aetiology, diagnostic pointers, and therapeutic measures. Postgrad Med J 76: 484, 2000.

-Marin JR, Trainor JL. Foreign body removal from the external auditory canal in a pediatric emergency department. Pediatr Emerg Care 22: 630, 2006.

-Marx JA, Hockberger RS, Walls RM. Rosen’s Emergency Medicine Concepts and Clinical Practice, 8e. Chapter 60.

-Roberts JR, Custalow CB, Thomsen, TW, Hedges JR. Roberts and Hedges’ Clinical Procedures in Emergency Medicine, 6e. Chapter 63.

-Tintinalli JE, Stapczynski JS, Ma OJ, Kline DM, Cydulka RK, Meckler GD, The American College of Emergency Physicians. Tintinalli’s Emergency Medicine, A Comprehensive Study Guide, 7e. Chapters 80, 119, 143.

-Triadafilopoulos G, Saltzman JR, Travis AC. Ingested Foreign Bodies and Food Impactions in Adults. www.uptodate.com

PEM Playbook – Big Labs, Little People: Troponin, BNP, D-Dimer, and Lactate

Originally published at Pediatric Emergency Playbook on April 1,
2016 – Visit to listen to accompanying podcast. Reposted with permission.

Follow Dr. Tim Horeczko on twitter @EMTogether

It’s a busy shift.  Today no one seems to have a chief complaint.

Someone sends a troponin on a child.  Good, bad, or ugly, how are you going to interpret the result?

And while we’re at it – what labs do I need to be careful with in children – sometimes the normal ranges of common labs can have our heads spinning!

Read on for bread-and-butter pediatric blood work and further, to answer the question – what’s up with troponin, lactate, d-dimer, and BNP in kids?


A fundamental tenet of emergency medicine:

We balance our obligation to detect a dangerous condition with our suspicion of the disease in given patient.

Someone with a cough and fever may simply have a viral illness, or he may have pneumonia.  Our obligation is to evaluate for the pneumonia.  It’s ok if we “miss” the diagnosis of a cold. It could be bad if we don’t recognize the pneumonia.

How do we decide?  Another fundamental concept:

The threshold.

Depending on the disease and the particular patient, we have a threshold for testing, and a threshold for treating.  Every presentation – and every patient for that matter – has a complicated interplay between what we are expected to diagnose, how much we suspect that particular serious diagnosis, and where testing and treating come into play.

What’s wrong with “throwing on some labs”?

Easy to do right?  They are but a click away…

Often a good history and physical exam will help you to calibrate your investigational thresholds.  This is especially true in children – the majority of pediatric ambulatory visits do not require blood work to make a decision about acute care.  If your patient is ill, then by all means; otherwise, consider digging a bit deeper into the history, get collateral information, and make good use of your general observation skills.

First, a brief word about basic labs.

The punchline is, use a pediatric reference.

If you don’t have a trusted online reference available during your shift, make sure you have something like a Harriett Lane Handbook accessible to you. Don’t rely on your hospital’s lab slip or electronic medical record to save you, unless you are sure that they use age-specific pediatric reference ranges to flag abnormal values. Believe it or not, in this 21st century of ours, some shops still use adult reference ranges when reporting laboratory values on children.

Notable differences in basic chemistries

Potassium: tends to run a bit higher in infants, because for the first year of life, your kidneys are inefficient in excreting potassium.

BUN and creatinine: lower in children due to less muscle mass, and therefore less turnover (and usually lack of other chronic disease)

Glucose: tends to run lower, as children are hypermetabolic and need regular feeding (!)

Alkaline phosphatase: is always high in normal, growing children, due to bone turn over (also found in liver, placenta, kidneys)

Ammonia: high in infancy, due to immature liver, trends down to normal levels by toddlerhood

ESR and CRP: low in healthy children, as chronic inflammation from comorbidities is not present; both increase steadily with age

Thyroid function tests: all are markedly high in childhood, not as a sign of disease, but a marker of their increased metabolic activity

Big Labs


Reliably elevated in myocarditis, and may help to distinguish this from pericarditis (in addition to echocardiography)

Other causes of elevated troponin in children include: strenuous activity, status epilepticus, toxins, sepsis, myocardial infarction (in children with congenital anomalies).  Less common causes of troponemia are: Kawasaki disease, pediatric stroke, or neuromuscular disease.

Don’t go looking, if you won’t do anything with the test.

Brain natriuretic peptide (BNP)

In adults, we typically think of a BNP < 100 pg/mL as not consistent with symptoms caused by volume overload.

Luckily, we have data in children with congenital heart disease as well.  Although each company’s assay reports slightly different cut-offs, in general healthy pediatric values match healthy adult values.

One exception is in the first week of life, when it is high even in healthy newborns, due to the recent transition from fetal to newborn circulation.

Use of BNP in children has been studied in both clinic and ED settings. Cohen et al. in Pediatrics used BNP to differentiate acute heart failure from respiratory disease in infants admitted for respiratory distress. They compared infants with known CHF, lung disease, and matched them with controls.

Later, Maher et al. used BNP in the emergency department to differentiate heart failure from respiratory causes in infants and children with heart failure and those with no past medical history.

The bottom line is:

BNP reliably distinguishes cardiac from respiratory causes of shortness of breath in children with a known diagnosis of heart failure.


To cut to the chase: d-dimer for use as a rule-out for pulmonary embolism has not been studied in children.

The only data we have in using d-dimer in children is to prognosticate in established cases. It is only helpful to track therapy for children who have chronic clots.

This is where our adult approach can get us into trouble. Basically, think of the d-dimer in children like it doesn’t even exist. It’s not helpful in our setting for our indications.   An adult may have an idiopathic PE – in fact, up to a third of adults with PE have no known risk factor, which makes decision tools and risk stratification important in this population.

Children with PE almost always have a reason for it.


There is at least one identifiable risk factor in up to 98% of children with pulmonary embolism. The majority have at least two risk factors.

If you’re suspecting deep venous thrombosis, perform ultrasonography, and skip the d-dimer.

If you’re worried about PE, go directly to imaging. In stable patients, you may elect to use MR angiography or VQ scan, but most of us will go right to CT angiography. Radiation is always a concern, but if you need to know, get the test.

This is yet another reminder that your threshold is going to be different in children when you think about PE – they should have a reason for it. After you have excluded other causes of their symptoms, if they have risk factors, and you are still concerned, then do the test you feel you need to keep this child safe.

You are the test.

Risk factors only inform you, and you’ll have to just pull the trigger on testing in the symptomatic child with risk factors.


A sick child with sepsis syndrome?

The short answer – yes.

In the adult literature, we know that a lactate level above 4 mmol/L in patients with severe sepsis was associated with the need for critical care. This has been studied in children as well, and an elevated lactate in children – typically above 4 – was a predictor of prolonged ICU course and mortality in septic patients.

The acute recognition and treatment of sepsis is first and foremost, clinical.

Our goal is to promote perfusion and provide oxygen to the tissues. Laboratory testing is not a substitute for clinical assessment – it should be used as an extension of your assessment.  There are two main reasons for an elevated lactate: the stress state and the shock state.

The stress state is due to hypermetabolism and an increase in glycolysis, as an example, in early sepsis. The shock state is due to tissue hypoxia, seen in septic shock. The confusion and frustration with lactate is that we often test the wrong people for it.

We could use it to track treatment, and see if we can clear the lactate; decreased lactate levels are associated with a better outcome in adults. Serial clinical assessments are even more useful to gauge your success with treatment.

We should use lactate to detect occult shock. Children compensate so well for shock, that subtle tissue hypoxia may not be detected until later. It may inform your decision for level of care, intensive care versus some other lower level.

Have you every been in this situation:

“Why, oh why, did we send a lactate?”

There are times when a lactate is ordered – maybe by protocol or maybe accidentally – or maybe in retrospect, the patient didn’t need it. Here is a quick mnemonic to remember the reasons for an elevated lactate: LACTATES


Lliver – any liver disease affects how lactate is metabolized by the Cori cycle
Aalbuterol (or for our international friends, salbutamol), beta-agonists like albuterol, increase lactate production via cyclic amp
C“can’t breathe” – respiratory distress and increased work of breathing shifts the ratio of aerobic and anerobic repiration
Ttoxins – all kinds of wonder drugs and recreational drugs do it – look up your patient’s list if you’re suspicious
Aalcohol, not an infrequent offender
Tthiamine deficiency – think of this in your cachectic or malnourished patients
Eepinephrine – a by-product of the Cori cycle, how lactate is metabolized. Difficult to interpret lactates when a patient is on an epinephrine drip.
Sseizure or shock – most commonly septic, but can be any type: cardiogenic, bstructive, hypovolemic, distributive.

Bottom line: high serum lactate levels have been associated with morbidity and mortality in children with sepsis and trauma, the two best-studied populations.

A summary of how labs can help you – or hurt you – in pediatric emergency medicine:

  1. Have a good reference for normal values and always be skeptical of how your lab reports them.
  2. Troponin testing is great for the child with suspected cardiogenic shock, myocarditis, or in unwell children with congenital heart disease.
  3. BNP in children can be used just like you do in adults – to get a sense of whether the presenting symptoms are consistent with heart failure.
  4. D-dimer is mostly a waste of time in the PED.
  5. Lactate can be useful in the right patient – use it to risk-stratify the major trauma patient or the patient with sepsis that may be suffering from occult shock.
  6. And lastly, make sure that you are mindful of your threshold for testing, and our threshold for treatment. If will vary by disease and by the patient at hand.



Gupta SK, Naheed Z. Chest Pain in Two Athletic Male Adolescents Mimicking Myocardial Infarction. Pediatr Emer Care. 2014;30: 493-495.

Kelley WE, Januzzi JL, Christenson RH. Increases of Cardiac Troponin in Conditions other than Acute Coronary Syndrome and Heart Failure. Clinical

Chemistry. 2009; (55) 12:2098–2112.

Kobayashi D, Aggarwal S, Kheiwa A, Shah N. Myopericarditis in Children: Elevated Troponin I Level Does Not Predict Outcome. Pediatr Cardiol. 2012; 33:1040–1045.

Koerbin G, Potter JM, Abhayaratna WP et al. The distribution of cardiac troponin I in a population of healthy children: Lessons for adults. Clinica Chimica Acta. 2016; 417: 54–56.

Liesemer K, Casper TC, Korgenski K, Menon SC. Use and Misuse of Serum Troponin Assays in Pediatric Practice. Am J Cardiol. 2012;110:284 –289.

Newby KL et al. for the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents. ACCF 2012 Expert Consensus Document on Practical Clinical Considerations in the Interpretation of Troponin Elevations. J Am Coll Cardiol. 2012; 60(23): 2427-2463.

Schwartz MC, Wellen S, Rome JJ et al. Chest pain with elevated troponin assay in adolescents. Cardiology in the Young; 2013. 23: 353–360.


Auerbach SR, Richmond ME, Lamour JM. BNP Levels Predict Outcome in Pediatric Heart Failure Patients Post Hoc Analysis of the Pediatric Carvedilol Trial. Circ Heart Fail. 2010;3:606-611.

Cohen S, Springer C, Avital A et al. Amino-Terminal Pro-Brain-Type Natriuretic Peptide: Heart or Lung Disease in Pediatric Respiratory Distress? Pediatrics. 2005;115:1347–1350.

Fried I, Bar-Oz B, Algur N et al. Comparison of N-terminal Pro-B-Type Natriuretic Peptide Levels in Critically Ill Children With Sepsis Versus Acute Left Ventricular Dysfunction. Pediatrics. 2006; 118(4): 1165-1168.

Koch A, Singer H. Normal values of B type natriuretic peptide in infants, children, and adolescents. Heart. 2003;89:875–878.

Maher KO, Reed H, Cuadrado A et al. , B-Type Natriuretic Peptide in the Emergency Diagnosis of Critical Heart Disease in Children. Pediatrics. 2008;121:e1484–e1488.

Mir TS, Marohn S, Laeer S, Eistelt M. Plasma Concentrations of N-Terminal Pro-Brain Natriuretic Peptide in Control Children From the Neonatal to Adolescent Period and in Children With Congestive Heart Failure. Pediatrics. 2002;110(6)1:6.

Mir TS, Laux R, Hellwege HH et al. Plasma Concentrations of Aminoterminal Pro Atrial Natriuretic Peptide and Aminoterminal Pro Brain Natriuretic Peptide in Healthy Neonates: Marked and Rapid Increase After Birth. Pediatrics. 2003;112:896–899.


Goldenberg NA, Knapp-Clevenger RA, Manco-Johnson MJ. Elevated Plasma Factor VIII and d-Dimer Levels as Predictors of Poor Outcomes of Thrombosis in Children for the Mountain States Regional Thrombophilia Group. Pediatrics. 2003;112:896–899.

Manco-Johnson MJ. How I treat venous thrombosis in children. Blood. 2006; 107(1)21-31.

Naqvi M, Miller P, Feldman L, Shore BJ. Pediatric orthopaedic lower extremity trauma and venous thromboembolism. J Child Orthop. 015;9:381–384.

Parasuraman S, Goldhaber SZ. Venous Thromboembolism in Children. Circulation. 2006;113:e12-e16.

Strouse JJ, Tamma P, Kickler TS et al. D-Dimer for the Diagnosis of Venous Thromboembolism in Children. N Engl J Med. 2004;351:1081-8.


Andersen LW, Mackenhauer J, Roberts JC et al. Etiology and therapeutic approach to elevated lactate. Mayo Clin Proc. 2013; 88(10): 1127–1140.

Bai et al. Effectiveness of predicting in-hospital mortality in critically ill children by assessing blood lactate levels at admission. BMC Pediatrics. 2014; 14:83.

Scott HF, Donoghue AJ, Gaieski DF et al. The Utility of Early Lactate Testing in Undifferentiated Pediatric Systemic Inflammatory Response Syndrome. Acad Emerg Med. 2012; 19:1276–1280.

Shah A, Guyette F, Suffoletto B et al. Diagnostic Accuracy of a Single Point-of-Care Prehospital Serum Lactate for Predicting Outcomes in Pediatric Trauma Patients. Pediatr Emer Care. 2013; 29:715-719.

Topjian AA, Clark AE, Casper TC et al. for the Pediatric Emergency Care Applied Research Network. Early Lactate Elevations Following Resuscitation From Pediatric Cardiac Arrest Are Associated With Increased Mortality. Pediatr Crit Care Med. 2013; 14(8): e380–e387.

This post and podcast are dedicated to Daniel Cabrera, MD for his vision and his leadership in thinking ‘outside the box’.


Troponin     |     BNP     |     D-Dimer     |     Lactate

Powered by #FOAMed — Tim Horeczko, MD, MSCR, FACEP, FAAP

Cystic Fibrosis: ED Management, Pearls and Pitfalls

Authors: Stephanie Tassin, MD (EM Resident at SAUSHEC) and Brit Long, MD (@long_brit, EM Attending Physician at SAUSHEC) // Edited by: Jennifer Robertson, MD, MSEd and Alex Koyfman, MD (@EMHighAK, EM Attending Physician, UTSW Medical Center / Parkland Memorial Hospital)


Cystic fibrosis (CF) is a life-shortening, autosomal recessive disease that affects multiple organ systems via mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) chloride channel. In short, a defective CFTR channel prevents mucous and exocrine glands from secreting chloride. Without chloride, water cannot follow, and the end result is thick, hyperviscous secretions in the lung, sinuses, pancreas, intestines, and biliary system [1].  In the sweat glands, chloride reabsorption is impaired, leading to excess sodium chloride loss. With excessive sweating, this can lead to hyponatremic hypochloremic dehydration. It also has detrimental effects on the reproductive, musculoskeletal, and urinary systems, but these are rarely relevant in the Emergency Department (ED) [2].

CF affects 1 in 3,200 Caucasian births and is primarily thought of as a Caucasian disease, though it is also seen in other ethnicities at lower frequencies. As of 2014, less than 2/3 of cases are detected by newborn screening, so providers must keep it on their differential, especially in young children with recurrent pulmonary infections, sinus infections, failure to thrive, and constipation or meconium ileus [2].

Case 1

A 22 year old female with cystic fibrosis presents to you complaining of increasing shortness of breath over the last week. Her chronic cough has gotten worse recently and it is productive of occasionally blood-tinged and green sputum. She reports low-grade fevers at home as well as fatigue, headache, and anorexia. She just moved to town and has no medical records in your system. Vital signs include blood pressure (BP) 110/64, heart rate (HR) 110, respiratory rate (RR) 34, oxygen saturation (SpO2) 92% on room air, temperature (T) 100.1°Farenheit (F) orally. Her examination is remarkable for coarse breath sounds with scattered expiratory wheezes, a slightly increased work of breathing, and clubbing of her fingernails. Her chest x-ray is shown below.



You put her on oxygen by nasal cannula and give her a small intravenous (IV) fluid bolus, but you wonder what to do next. You do not see a lot of patients with CF, but you see a lot of chronic obstructive pulmonary disease (COPD), and she looks almost exactly like a patient with a COPD exacerbation. Do you just treat her like COPD with nebulizer treatments, steroids, antibiotics, and bi-level positive pressure ventilation (BiPAP)? What antibiotics do you use? Is there anything else you can offer?

CF Pulmonary Exacerbations

Progressive pulmonary disease accounts for 85% of the mortality in CF[3]. From a young age, the CF patient is colonized with a predictable spectrum of bacteria that persist and cause chronic infection and inflammation [1]. Typically, this causes a persistent, productive cough and an obstructive pattern on pulmonary function tests. This may progress to chronic bronchitis and bronchiectasis with occasional exacerbations. These pulmonary exacerbations are generally characterized by increasing dyspnea, tachypnea, changes in sputum production, increased adventitious lung sounds, malaise, anorexia, and a decrease in pulmonary function [3, 4, 5].

The majority of pulmonary exacerbations are caused by clonal expansion of existing strains of bacteria, rather than an acquisition of new bugs [6]. Still, other causes of pulmonary distress must be kept on the differential in cystic fibrosis patients. Allergic bronchopulmonary aspergillosis (ABPA) should be suspected in patients with significant wheezing. Though it will likely not be diagnosed in the emergency department [6], it is reasonable to start steroids in these patients as discussed below. Spontaneous pneumothorax can occur, especially in older patients with advanced disease. Recurrence is also common [7].  Minor hemoptysis is also common in CF, especially during a pulmonary exacerbation. Beyond checking the international normalized ratio (INR) to rule out any contributing vitamin K deficiency, this generally only requires reassurance. A complete blood count (CBC) is rarely needed unless in circumstances concerning for anemia.

In patients with suspected pulmonary exacerbations, plain chest radiographs should be ordered to exclude pneumothorax. Other findings such as mucous plugging, peribronchial thickening, and air space disease are common but not specific for an acute exacerbation [8].


Early in life, the most common bugs cultured from the lungs are Staphylococcus aureus and non-typeable Haemophilus influenzae [9]. Pseudomonas aeruginosa is also often isolated early and is the most significant pathogen in CF. Once established, P. aeruginosa is essentially impossible to eradicate due to a combination of genetic adaptations, biofilm formation, and an optimal environment in CF airways.  Burkholderia cepacia complex is less common, but is still occasionally seen and can rapidly lead to necrotizing pneumonia, sepsis, and death. Other common organisms include S. maltophilia, and A. xylosoxidans, which tend to be less virulent.  Aspergillus spp. is isolated from more than 25% of patients but it rarely causes invasive infection outside of the immunocompromised post-transplant patient. However, allergic bronchopulmonary aspergillosis (ABPA) can cause significant illness and it is a diagnosis to keep in mind if your patient presents with significant wheezing [9].


Early recognition and treatment of pulmonary exacerbations has been associated with slower long-term decline in lung function [10]. Pulmonary exacerbations are treated with antibiotics and supportive measures aimed at airway clearance. In cases of ABPA, which is usually not diagnosed in the ED, patients may present with asthma-type symptoms and are usually treated with steroids [9].

Management is summarized in Table 1.

 Table 1

Management Considerations in CF Pulmonary Disease
1. Antipseudomonal antibiotics (high doses): Aminoglycoside + β-lactam

2. Bronchodilator – either MDI or nebulized albuterol

3. Nebulized 7% saline, 4mL

4. BiPAP if needed

5. Only use steroids if there are significant asthma-type symptoms (i.e. wheezing)


In general, antibiotic choices should be tailored to the patient’s previous culture results if known. For mild exacerbations or presumed viral upper respiratory infections not requiring inpatient admission, it is reasonable to discharge patients on an oral antibiotic such as amoxicillin-clavulanate that will cover both H. influenza and S. aureus. If the patient has a known history of Pseudomonas infection, an anti-pseudomonal fluoroquinolone such as ciprofloxacin may be used (1 month – 5 years: 15mg/kg max 750mg/dose BID, 5-18 years: 20mg/kg BID). Recommended duration of treatment is 2 weeks [5]. Patients should continue to use their regular nebulized anti-pseudomonal antibiotic. In these cases, sputum should be sent for culture prior to starting therapy. If there is no improvement on oral antibiotics, there should be a low threshold to admit for intravenous treatment [6].

For more severe exacerbations or those failing outpatient therapy, treatment is usually aimed at Pseudomonas unless the previous cultures identify a different pathogen as the likely culprit. For presumed Pseudomonas infection in a patient with a severe exacerbation, double antibiotic therapy is preferred with two anti-pseudomonal drugs with different mechanisms of action. This is most often accomplished with an aminoglycoside and β-lactam [5]. While results of previous cultures are useful to determine which bug is most likely causing an exacerbation, antibiotic sensitivities for Pseudomonas in particular are not useful for selecting antibiotics and have no effect on patient outcomes [6, 11].

Of the aminoglycosides, tobramycin is most frequently used and has been extensively studied [12, 13]. Amikacin is also used but is used less often. Gentamicin is avoided in CF due to an increased risk of nephrotoxicity [12].  The CF Foundation recommends that aminoglycosides be administered in once daily doses rather than TID to maximize efficacy and minimize nephrotoxicity [3]. Of the β-lactams, ceftazidime is most commonly used among accredited CF centers, followed by cefepime, piperacillin-tazobactam, meropenem, ticarcillin-clavulanate, and aztreonam [14].

Note that patients with CF require larger doses of antibiotics (often larger doses than are FDA-approved) due to a larger volume of distribution, increased renal clearance, and the increased minimal inhibitory concentration (MIC) of Pseudomonas [12, 15]. Many providers, even in CF Foundation-accredited care centers, continue to prescribe inadequate doses of anti-pseudomonal antibiotics despite published dosing guidelines [16]. The best available evidence, based on the pharmacokinetics, pharmacodynamics, tolerability, and efficacy of different regimens, supports the dosing regimens listed below in Table 2 [15].

Table 2 – Antibiotic Choices in CF Exacerbation

Aminoglycoside Dose
Tobramycin 10 mg/kg/day in one daily dose
Amikacin 30-35 mg/kg/day in one daily dose
β-lactam Dose
Ceftazidime 200-400 mg/kg/day div every 6-8 hr (max 8-12 g/day)
Cefepime 150-200 mg/kg/day div every 6-8 hr (max 6-8 g/day)
Pip-tazo 350-600 mg/kg/day div Q4H (max 18-24 g/day piperacillin)
Meropenem 120 mg/kg/day div Q8H
Ticarcillin-clavulanate 450-750 mg/kg/day Q6H (max 24-30 g/day ticarcillin)
Aztreonam 200-300 mg/kg/day div Q6H (max 8-12 g/day)

For infections caused by bugs other than Pseudomonas, culture and susceptibility testing usually guide antibiotic choice. Staphylococcus aureus is a common pathogen in CF and can be treated according to local resistance patterns.  Other typical bugs, notably B. cepacia complex, S. maltophilia, and A. xylosoxidans, tend to be very antibiotic-resistant, so treatment should be guided by culture and susceptibility testing [17].

Patients with CF have variable responses to bronchodilators. Approximately 50% of these patients will have some degree of bronchial hyperresponsiveness [9]. Given the relatively benign side effect profile of beta-agonists, nebulized treatments in the ED may assist in airway opening. Bronchodilators may not be the mainstay of treatment of pulmonary exacerbations, but it is helpful when administered prior to treatment with nebulized hypertonic saline, as discussed below.

Mucolytics (hypertonic saline)

Inhaled hypertonic saline (HS) has been shown to improve the properties of sputum and acutely increase mucociliary clearance in patients with CF. Unlike the other major mucolytic used for CF called DNase I (Dornase alpha), HS appears to be beneficial during an acute exacerbation, especially when followed by chest physiotherapy [9, 10]. A nebulized dose of 4mL of 7% saline has been shown to improve symptoms and lung function when compared to a control treatment with 0.12% saline. Patients treated with the 7% saline nebs during their hospitalization are more likely to return to their pre-exacerbation FEV1 with a number needed to treat of 6 [10]. It is generally well tolerated, but it does have the potential to cause a transient airflow obstruction. For this reason, patients should be treated with a bronchodilator immediately prior to hypertonic saline [18].

Positive pressure ventilation

As in COPD, non-invasive positive pressure ventilation is an attractive treatment option for severe pulmonary exacerbations. Several observational studies have looked at the use of NPPV in these situations and concluded that it may be useful, especially if used early during an acute exacerbation. It is at the very least preferable to invasive ventilation due to the high mortality rates seen in patients with CF who get intubated[19].


Progression of lung disease in CF is largely mediated by chronic inflammation, so it makes sense that corticosteroids should provide some benefit [20].  Unfortunately, although treatment of CF with long-term corticosteroids has shown some improvement in lung function, the risks and significant side effect profile preclude their utility on a regular basis. Theoretically, short-term use of steroids during an acute pulmonary exacerbation should have a more acceptable risk-benefit profile. Only two small studies (n=44) have looked at the use of steroids during an acute exacerbation and showed a small trend towards improvement in lung function at follow up [20, 21]. In the larger of the studies, 3 of the 12 patients in the prednisone group had to be withdrawn from the study either from hypertension or hyperglycemia [20]. The CF Foundation concludes there is insufficient evidence to recommend routine use of steroids during an acute exacerbation [3].  Though they are not recommended for routine use, they may provide more benefit in patients with predominant asthma-type symptoms or suspected ABPA [9]. Close consultation with the patient’s pulmonologist if possible is needed to discuss steroid treatment.

Your patient reports growing Pseudomonas from multiple cultures in the past, so you decide to treat her with cefepime and tobramycin. You also give her an albuterol treatment immediately followed by a 7% saline neb. You had considered BiPAP, but her respiratory rate and work of breathing improve following treatment. She is admitted to the hospital for continued IV antibiotics and airway clearance therapy.

Case 2

A 12 year old male with cystic fibrosis presents with 3 weeks of progressively worsening crampy right lower quadrant (RLQ) abdominal pain and now non-bilious vomiting and oral fluid intolerance. He had a few episodes of diarrhea yesterday but has since had no bowel movement. He takes pancreatic enzyme replacement therapy and has chronic constipation, for which he uses polyethylene glycol (PEG) 3350 daily. His vital signs are normal for his age except for a heart rate of 120 beats per minute (bpm). His examination is significant for dry mucous membranes and abdominal distention with RLQ tenderness, but no rigidity or guarding. You think you feel a small mass in the RLQ. His abdominal x-ray is shown below:


Distal Intestinal Obstruction Syndrome

Distal Intestinal Obstruction Syndrome (DIOS), previously known as “meconium ileus equivalent,” is an entity unique to CF that is caused by partial or complete obstruction of the ileocecum by inspissated fecal material [22]. It is seen in all age groups, though it is more common in adults and is almost always seen in patients with pancreatic insufficiency [23].


DIOS commonly presents with progressive, cramping abdominal pain that usually located in the RLQ or peri-umbilical region. Pain may be acute, but it often precedes the actual obstruction by several weeks or even months. Patients may have abdominal distension first, however.  Though DIOS is commonly seen with constipation, it may also be seen with diarrhea or even normal bowel movements [23]. On examination, the inspissated material can usually be felt in the RLQ, although a palpable mass may be present for years without causing obstructive symptoms [24].


A plain abdominal radiograph is the first line imaging test that should be obtained. It will typically show an accumulation of “bubbly” or “granular” fecal material in the distal ileum [23]. The triad of characteristic abdominal pain, palpable RLQ mass, and distal ileal fecal material on x-ray is usually sufficient to diagnose DIOS. If symptoms or radiographic findings are atypical, or if there is no improvement with treatment, additional imaging such as ultrasound or CT scan should be obtained to evaluate mimics such as appendicitis or intussusception [24].

Differential Diagnosis

It may be difficult to distinguish impending DIOS from chronic constipation by history alone. Constipation usually has a more gradual onset of symptoms with fecal material distributed throughout the colon, as opposed to being localized to the right lower quadrant [22]. However, these entities often coexist and except in severe cases, initial treatment is the same.

Appendicitis should also be on the differential, but unfortunately is sometimes difficult to evaluate. Chronically inspissated mucoid contents may lead to a distended appendix that is difficult to distinguish from an acutely inflamed appendix. This can often lead to a delay in diagnosis of appendicitis, resulting in increased rates of appendiceal perforation and abscess formation in patients with CF.  Other causes of bowel obstruction must also be considered such as adhesions, malignancy, or intussusception. Intussusception occurs in about 1% of CF patients and is a common mimic but can also be a complication of DIOS [24].


First, correct any fluid or electrolyte abnormalities and treat any associated infections. After that, treatment is fairly straightforward and involves laxatives administered either orally, by nasogastric tube, or by enema. Patients with incomplete obstructions usually respond to oral therapy with any PEG bowel prep solution such as GoLytely®, Klean-Prep®, or Movicol®. Another option for oral therapy is Gastrografin diluted in water or juice (50mL in 200mL for kids under 6 years, and 100mL in 400mL for everyone else). Therapy is continued until symptoms resolve and bowel movements are clear. Thus, admission may be required [24].
For patients with complete obstruction, PO intolerance, or failure of oral therapy, a nasogastric tube should be placed for decompression, followed by a Gastrografin enema. Since Gastrografin is radiopaque, these enemas can be both diagnostic and therapeutic and may be used to monitor progress [23, 24, 25]. However, these enemas can cause significant fluid shifts as well as intestinal ischemia, perforation, and necrosis, so they should only be administered by an experienced radiologist. These patients all require admission with a low threshold for surgical consultation [24, 26].
You suspect an incomplete obstruction, but he remains PO intolerant. You place an IV to draw labs and give him a 20 cc/kg bolus of LR. After correcting his electrolytes, you place a nasogastric tube for decompression and to administer GoLytely for bowel irrigation. He has a small bowel movement and slight improvement in pain. He is admitted to pediatrics for continued bowel irrigation.


-CF is not limited to Caucasians and over 33% of cases are missed by newborn screening programs [2].

-Pulmonary exacerbations must be treated early and aggressively to slow the decline in lung function [10].

Treatment of pulmonary exacerbations:

  1. Antipseudomonal antibiotics (high doses): Aminoglycoside + β-lactam
  2. Bronchodilator – either MDI or nebulized albuterol
  3. Nebulized 7% saline, 4mL
  4. BiPAP if needed’
  5. Only use steroids if there are significant asthma-type symptoms (i.e. wheezing)


-Diagnosed by classic history, mass in the RLQ, and localized fecal material on plain abdominal film [22].

-Don’t miss appendicitis or intussusception. Get an ultrasound and/or CT scan if needed [24].

-Correct fluids and electrolytes first [24].

-If PO tolerant, may treat from above with PEG solution (e.g. GoLytely®) or Gastrografin diluted 1:4 in water or juice. Patients may require an NG tube in order to consume enough of the laxative [24].

-If PO intolerant or with bilious vomiting (i.e. complete obstruction), decompress the stomach with a nasogastric tube. Call radiology for a Gastrografin enema [24].

-Treat until bowel movements are clear and watery [24].


References / Further Reading

  1. Rowe SM, Miller S, Sorscher EJ. Cystic Fibrosis. N Engl J Med 2005; 352:1992-2001.
  2. Cystic Fibrosis Foundation Patient Registry: Annual Data Report to the Center Directors, 2014. https://www.cff.org/2014_CFF_Annual_Data_Report_to_the_Center_Directors.pdf/ (Accessed on June 30, 2016).
  3. Flume PA, Mogayzel PJ, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Respir Crit Care Med. 2009 Nov 1;180(9):802-8.
  4. Bilton D, Canny G, Conway S, et al. Pulmonary exacerbation: towards a definition for use in clinical trials. Report from the EuroCareCF Working Group on outcome parameters in clinical trials. J Cyst Fibros 2011; 10: Suppl. 2, S79-S81.
  5. Smyth A, Elborn JS. Exacerbations in cystic fibrosis: 3 – Management. Thorax 2008; 63: 180-184.
  6. Bhatt JM. Treatment of pulmonary exacerbations in cystic fibrosis. Eur Respir Rev. 2013 Sep 1;22(129):205-16. PMID 23997047.
  7. Flume PA. Pneumothorax in cystic fibrosis. Current Opinion in Pulmonary Medicine 2011. 17(4):220-225.
  8. Greene KE, Takasugi JE, Godwin JD, et al. Radiographic changes in acute exacerbations of cystic fibrosis in adults: a pilot study. Am J Roentgenol 1994; 163(3):557-62.
  9. Gibson RL, Burns JL, Ramsey BW. Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis. Am J Respir Crit Care Med 2003; 168:918-951.
  10. Dentice, RL, Elkins MR, Middleton PG, et al. A randomized trial of hypertonic saline during hospitalization for exacerbation of cystic fibrosis. Thorax 2016; 71:141-147.
  11. Hurley MN, Ariff AH, Bertenshaw C, et al. Results of antibiotic susceptibility testing do not influence clinical outcome in children with cystic fibrosis. J Cyst Fibros 2012; 11:288-292.
  12. Talwalkar JS, Murray TS. The Approach to Pseudomonas aeruginosa in Cystic Fibrosis. Clin Chest Med 2016; 37:69-81.
  13. Young DC, Zobell JT, Stockmann C, et al. Optimization of Anti-Pseudomonal Antibiotics for Cystic Fibrosis Pulmonary Exacerbations: V. Aminoglycosides. Pediatric Pulmonology 2013; 48:1047-1061.
  14. Fischer DR, Namanny H, Zobell JT. Follow-up survey of the utilization of anti-pseudomonal beta-lactam antibiotics at U.S. cystic fibrosis centers. Pediatr Pulmonol. 2016 Jul; 51(7):668-9.
  15. Zobell JT, Young DC, Waters CD, et al. Optimization of Anti-Pseudomonal Antibiotics for Cystic Fibrosis Pulmonary Exacerbations: VI. Executive Summary. Pediatric Pulmonology 2013; 48:525-537.
  16. Zobell JT, Waters CD, Young DC, et al. Optimization of Anti-Pseudomonal Antibiotics for Cystic Fibrosis Pulmonary Exacerbations: II. Cephalosporins and Penicillins. Pediatric Pulmonology 2013; 48:107-122.
  17. Doring G, Flume P, Heijerman H, et al. Treatment of lung infection in patients with cystic fibrosis: Current and future strategies. Journal of Cystic Fibrosis 11 (2012):461-479.
  18. Elkins MR, Robinson M, Rose BR, et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 2006; 354:229.
  19. Fauroux B. Why, when and how to propose noninvasive ventilation in cystic fibrosis? Minerva Anestesiol 2011; 77:1108-1114.
    20. Dovey M, Aitken ML, Emerson J, McNamara S, Waltz DA, Gibson RL. Oral corticosteroid therapy in cystic fibrosis patients hospitalized for pulmonary exacerbations: a pilot study. Chest 2007;132:1212-1218.
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