Post ROSC care

Cite this article as:
Costas Kanaris. Post ROSC care, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.29109

Or some pointers on clinical management following the successful return of spontaneous circulation in children. 

It’s 5:40 am at Bubblesville ED. The red phone rings. The paramedic crew informs you that they are five minutes away with a 7 kg, 6-month-old, previously thriving, baby boy called Tarquin. He has had a witnessed out of hospital cardiac arrest at home and there was no prodromal illness according to the family. He choked during a breastfeed, turned blue, and stopped breathing. He had 5 minutes of CPR by the parents by the time the ambulance arrived and had ROSC by the paramedic team after a further 8 minutes. The rhythm strip was consistent with a PEA arrest. They are hand-ventilating him through an LMA.

This is his capillary gas on arrival in the ED
  1. What are your clinical priorities?
  2. What clinical problems do you anticipate in the immediate post-arrest phase?
  3. Who do you call for help?
  4. What do you do with the family whilst you’re managing the patient?
  5. What investigations do you need?

A systematic, collaborative, well-led approach to advanced paediatric life support can maximize the chances of the clinical team achieving a return of spontaneous circulation in a child that has arrested. We’ve seen the drill be before. We can go through our algorithms expertly and the 5-H’s and 5-T’s roll off the tongue, even under duress. The return of a pulse is heralded as the “hallelujah moment”, almost as if the patient is now healthy and safe and all we have to do is wait for the paediatric critical care retrieval team to arrive.

Whilst traditional APLS teachings are vital for the dissemination of knowledge and it’s application in everyday clinical life, their main focus is on the initial phase of achieving a pulse with very little attention placed on the all-important post-resuscitation phase.  This part of care is crucial, not only if we are to minimize secondary brain injury to the child but also to improve the chances of permanent returns of spontaneous circulation. Good short and long term outcomes rely heavily on how well we manage the post-resuscitation stage. 

There are four phases of cardiac arrest:-

Phase one: Prevention. This is the pre-arrest phase. Child safety and injury prevention strategies are in place to recognize deterioration. Adequate monitoring by using early warning systems and a pro-active approach to management is likely to contribute to avoiding an arrest.

Phase two: No flow arrest. This is a period of cardiac arrest prior to us commencing CPR. Our aim here is to minimize the time it takes to start life support. It is key that we involve the cardiac arrest team quickly, we start chest compressions early, and that we do not delay defibrillation if this is needed. 

Phase three: Low flow resuscitation. This phase describes when CPR is in progress. The aim is to achieve high-quality CPR in order to allow adequate coronary and cerebellar perfusion. Maintaining good ventilation and oxygenation whilst avoiding aggressive over-ventilation is paramount. It is during this phase that we systematically approach and threaten the reversible causes of cardiac arrest. 

Phase four: This is the post-resuscitation phase after ROSC has been achieved. Our aim here is to optimize coronary and cerebral perfusion. Neuroprotection and treatment of arrhythmias as well as treatment of post-cardiac arrest syndrome come under this phase. 

Adult v paediatric arrest: What’s the difference?

Out of hospital cardiac arrests in children >16 years of age are relatively rare – reported at 8-20/100000/ year. The incidence is only comparable to that of the adult population, estimated at 70/100000/year. The incidence of in-hospital paediatric arrests is much higher, with a nearly one-hundred-fold increase, compared to the out-of-hospital incidence for the <16’s. 

Survival, and especially a “good” survival from a neurological perspective still remain poor. Out of hospital survival rates are estimated to be 5 – 12%. Only 0.3 to 4% of those that survive have no long-term neurological insult.

Children have cardiac arrests due to severe respiratory insult or circulatory collapse, in the main. Either can lead to a respiratory arrest coupled with hypoxia, which then results in a cardiac arrest. The overwhelming majority of cardiac arrests present with a non-shockable rhythm.  It is also worth noting that almost half of the paediatric population that have a cardiac arrest have other chronic comorbidities such as respiratory conditions e.g. asthma, congenital cardiac disorders, or neurodisability. 

In the adult population, cardiac arrest is more likely due to long-term comorbidities such as ischaemic heart disease. This contributes to the development of an acute myocardial insult and (usually) a shockable rhythm. Understanding the difference in pathology leading to a cardiac arrest between adults and children is vital. Reversing the cause of the respiratory compromise can make the impalpable pulse palpable, allowing us to perfuse our patient once again. 

The recommended CPR ratio of 15:2 for children aims to provide adequate ventilation for oxygenation as well as satisfactory cardiac compressions to maintain sufficient perfusion of the coronary and cerebral circulation. Adult studies looking at compression-only CPR in patients with VF arrest have shown that success in achieving ROSC is due to pre-existing pre-arrest aortic blood oxygen and pulmonary oxygen stores.  As a mere 14% of cardiac arrests are due to a shockable rhythm, combining ventilation and compressions is vital. 

What is the Post Cardiac Arrest Syndrome (PCAS)? 

PCAS describes the period in which our patients are at the highest risk of developing ventricular arrhythmias and reperfusion injuries after ROSC. This is secondary to prolonged ischaemia then reperfusion of vital organs, primarily the myocardium and central nervous system. Its systemic effects are not dissimilar to those encountered in severe sepsis. There are four stages to PCAS:

  • Immediate post-arrest – First 20 minutes. 
  • Early post-arrest – 20 minutes to 6-12 hours. 
  • Intermediate phase – 6-12 hours up to 72 hours.
  • Recovery phase – From 72h onwards. 

Neuroprotection

Even high-quality closed-chest CPR can only achieve 50% of normal cerebral blood flow at best. It is not a secret that the brain does not tolerate hypoxia or ischaemia, the effects on both of these processes are exponential during a cardiac arrest, the longer the downtime, the worse the neurological hit

The pathophysiological cascade for neurodegeneration following cardiac arrest is complex and multi-factorial. Following a hypoxic or ischaemic period the brain develops cerebral oedema and cerebral hyperaemia. There is impaired cerebral vascular reactivity and like any other organ trying to reperfuse, the post-ischaemic biochemical cascade is activated. All these factors contribute to a secondary brain injury. Of course, the duration of hypoxia will in large part dictate how severe the primary brain injury is and whether the patient is likely to survive or not. Brain injury can manifest as myoclonus, stroke, seizures, coma, or brain death. 

We can minimize the extent of secondary brain injury with simple proactive, neuroprotective measures:

  • Strict normothermia
  • Aggressive seizure prophylaxis
  • Avoiding hypoxia and hyperoxia
  • Tight circulatory monitoring and support
  • Patient position
  • Eucapnia and normoventilation
  • Vigilant glucose monitoring
  • Frequent neurological assessment, especially before the administration of anaesthetic agents and paralysis

Strict normothermia

Therapeutic hypothermia following a cardiac arrest during the intermediate phase (after VF in adults), as well as newborns with birth asphyxia, has shown some correlation with better neurological outcomes and reduced neurodisability.  Similarly, there is strong evidence linking core temperature above 38° with worse neurological outcomes in patients following cardiac arrest. There is a wide variation in practice in relation to therapeutic hypothermia.  Mild hypothermia after paediatric cardiac arrest is in the policy of some PICU’s. Patients are cooled to 33-34°C for 1 – 2 days and are then gradually rewarmed. Paralysis can be used as an adjunct to stop shivering. Temperatures below 32°C should be avoided as they are associated with worse survival, immunosuppression, arrhythmias, coagulopathies, and infections.  The decision to “cool” must be made early and in conjunction with your critical care transport team. You have many tools at your disposal to achieve this such as cold IV fluids, cooling blankets, and catheters. 

What is, and should be, more aggressively targeted is strict normothermia (temperatures between 36-37°C), and depending on local practice hypothermia can be targeted to 33-36°C. Avoidance of pyrexia is crucial. Fever can result in an increased metabolic demand of the brain. This contributes to more ischemic injury and more infarcts as the threshold for ischemia in the injured brain is lower than that of the normal brain. The brain can no longer auto-regulate the mismatch between cerebral blood flow and metabolic demand.

Aggressive seizure prophylaxis

Seizures after paediatric cardiac arrest can occur in up to 47% of cases. 35% of these can lead to refractory status epilepticus.  Whilst CFAM/EEG monitoring is unlikely to be available in your local PED, it is important to have a low threshold to administer a long-acting anti-epileptic or a continuous infusion of a short-acting medicine to prevent/avoid this from happening. Ideally, a continuous infusion of midazolam +/- levetiracetam (less arrhythmogenic than phenytoin but both will work) and standard national guidelines should be followed. 

Clues as to whether a patient is still fitting include:

  • Unexpected changes in the pupillary size (beware of the child that had atropine on induction with the “fixed dilated pupils”).
  • Sudden changes in BP or heart rate.  

If you have given a paralytic for intubation, do not fall into the trap of thinking that the patient is not seizing, only an EEG or CFAM can tell you that. It is better to err on the side of caution.

Avoiding hypoxia and hyperoxia

Avoiding hypoxia and hyperoxia are also key components in minimizing secondary brain injury.  Whilst hypoxia will further exacerbate secondary brain injury, hyperoxia  (PaO2 > 40 kPa) is also be associated with worse survival due to oxygen free-radical formation that can inactivate intracellular enzymes, damage DNA, and destroy lipid membranes. It is reasonable to have high concentration oxygen therapy during the low-flow resuscitation and early post-resuscitation phases (as the commonest causes are respiratory). In the subsequent phases, we should target oxygen saturations between 94 and 96% and be proactive in how we reduce the FiO2 whilst avoiding hypoxia. There is a caveat in cases of severe anaemia or carbon monoxide poisoning. Then it is clinically appropriate for the highest concentration of oxygen to be administered.

Tight circulatory monitoring and support

Inotropic support may also be needed early. A degree of myocardial dysfunction/stunning is expected following CPR. To ensure adequate cerebral perfusion we need to target an age-specific, physiologically normal blood pressure. Both hypo and hypertension can exacerbate secondary brain injury. Because of this, monitoring the blood pressure through an arterial line is preferred. If the local set-up or skillset does not allow for arterial line placement, especially in smaller children, having non invasive blood pressure on 1-2 minute cycles can be a useful proxy.  

The paediatric myocardium is much more resilient than its adult counterpart.  If the arrest is not secondary to congenital heart disease the paediatric heart can regain normal function within 12-24 hours.  During the first 20 minutes following ROSC poor cardiac function is due to profound systemic vasoconstriction and cellular acidosis. We can support the myocardium by supplying adequate fluid resuscitation, targeting normal (age-appropriate) blood pressure and inotropic support. Point of care ultrasound, CVP monitoring, or assessing for hepatomegaly/rales if there is no access to the former, can help us prevent fluid overload

Inotrope choice is usually made with the help of the critical care team and depends on the balance between the need for inotropy and vasoconstriction.  Adrenaline is preferred for inotropy, noradrenaline for vasoconstriction.  Be aware that severe acidosis can cause catecholamine resistance, so giving some bicarbonate if the pH <7 may help your inotropes work better. Routine administration of bicarbonate has not been shown to improve clinical outcomes. There are some special circumstances in which we should consider its use such as cases of hyperkalaemia or hypermagnesaemia and arrests due to tricyclic antidepressant overdose. 

Patient position

The patient position that can achieve optimum cerebral perfusion is with the patient semi-sat up at a 30-45 degree angle.

Eucapnia and normoventilation

Avoidance of hypercapnia or hypocapnia is important in preventing secondary brain injury. It is, therefore, recommended that eucapnia is achieved by targeting a PaCO2 between 4.5 and 5.5 kPa. Hyperventilation can cause hypoxia and increase intracranial pressure due to hyperaemia, it can also cause further cerebral ischemia. As the intrathoracic pressures increase, cardiac venous return is impaired. Since the myocardium is already injured this can have catastrophic effects causing the BP to plummet and subsequently impair cerebral perfusion.

Vigilant glucose monitoring 

Following ROSC, children are also at risk of developing hypoglycemia (glucose <3 mmol/L). There is good evidence to suggest that hypoglycaemia negatively impacts neurological outcome and cause hypoglycaemic seizures, especially in the younger ages. Vigilant glucose monitoring and correction as per APLS guidelines is important. If regular dextrose boluses are needed, consider a continuous glucose infusion. If the patient mounts an adequate stress response, they may become hyperglycaemic.  There is no evidence to suggest that aggressive glucose control with insulin in the non-diabetic patient is beneficial; wait with watchful deliberation and the glucose will usually return to normal levels with no intervention.

Frequent neurological assessment

It is important to frequently assess neurological status frequently after ROSC as this can help us prognosticate. Take the time to do a very quick assessment ideally before the administration of anaesthetic agents and paralysis. Document clearly pupillary size/reactivity, GCS (and its break down) and any respiratory effort or gasping. 

Adjunctive investigations

Following ROSC a number of investigations will be needed to guide diagnosis and therapy. Routine bloods such as renal function, electrolytes, liver function tests, full blood count, and clotting are a basic standard. In cases of lactaemia and/or severe metabolic acidosis ammonia and toxicology is useful. Arterial blood sampling is invaluable to allow quick correction of any electrolyte abnormalities and help titrate ventilation settings and (in part) guide inotropic support. Arterial samples will also help uncover any exposure to carbon monoxide, especially in burns cases. 

From an imaging perspective, a chest X-ray is vital in ascertaining tube positioning and lung pathology as well as cardiac contours in case a congenital or acquired heart disease is suspected. Head CT is obviously useful in cases in keeping with traumatic arrest and NAI but timing of the CT and whether it should take place pre-departure to PICU or after depends largely on local trauma network protocols so should ideally be discussed with the regional trauma team lead and paediatric critical care transport team. 

Children that die or arrest unexpectedly in the UK are subject to a sudden unexpected death in infancy investigation (SUDI) so the appropriate referrals need to be made to the child protection team, police and social care. It is important to clarify that even near-miss cases merit triggering the same SUDI process to ensure that any NAI cases don’t slip through the net. 

Transport pearls

After ROSC the patients will need stabilisation and transfer to PICU for on-going management. Depending on the geographical location of your hospital and the availability of a critical care retrieval service you may have to transfer the patient yourselves or look after them until he/she is retrieved by transport team. A good transport and adequate neuroprotection can be achieved by applying these simple pearls: 

  1. Aggressive temperature monitoring and control between 33°C and 37°C.
  2. Monitor for seizures and pre-empt with long-acting antiepileptic accordingly.
  3. Correct electrolytes and hypoglycaemia and monitor frequently.
  4. Nurse the patient a 45° degree angle.
  5. Aim for a higher end of normal BP and use inotropes to achieve this. If you can’t insert an arterial line, have the NIVBP cycle every couple of minutes. 
  6. In cases of trauma, blood products should be used for volume. In an atraumatic arrest, balanced solution boluses are less harmful than 0.9% saline; don’t forget that you are still likely to need blood products. 
  7. Aim for a pCO2 of 4.5-5.5 kPa; use your continuous EtCO2 monitor to titrate ventilation. 
  8. Vigilant and through history/examination to rule out NAI. Free up a member of the team to do a thorough history from the family, always suspect NAI until proven otherwise especially in children under 6 months. 
  9. Know your anaesthetic drug side-effects (atropine dilates pupils for example so impairs our ability to monitor for seizures). Primum non nocere. 
  10. Intraosseous access can be used instead of a central line, have a low threshold to insert one and do it early.
  11. Have a member of the team check-in with the family every 10-15 minutes to explain what is happening, this is a bad day at work for you but probably the worst day of their lives. 

Conclusion

Achieving ROSC is an important step to give our patients a shot at survival. In some cases, achieving ROSC can only give us enough time to prognosticate and understand that survival is not possible. In some other cases ROSC can be the stepping-stone for a good, meaningful survival with a good quality of life. To achieve that, we must be able to apply good quality post–ROSC care and aggressive, pre-emptive neuroprotection. Learn the PCAS disease process to beat the PCAS disease process.  The APLS algorithm has become the bread and butter of anyone that is involved in paediatric care. Understanding and applying the principles of post-cardiac arrest syndrome is equally vital in improving survival outcomes for our patients. Learn the pearls, use them, teach them and I guarantee that it will make a difference.

The Travelling Doctors Suitcase: Fiona Reilly at DFTB19

Cite this article as:
Team DFTB. The Travelling Doctors Suitcase: Fiona Reilly at DFTB19, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.20048
Fiona Reilly is many things as we found out at DFTB17. In this talk from the final plenary session of DFTB19 she takes us on a journey – from her early days working in rural Australia to now, working at a big city hospital – and she reminds us that there are always lessons we can learn along the way. Showing up can be hard, but with a little extra care, we can be there for our patients.
This talk was recorded live at DFTB19 in London, England. With the theme of  “The Journey” we wanted to consider the journeys our patients and their families go on, both metaphorical and literal. If you want our podcasts delivered straight to your listening device then subscribe to our iTunes feed or check out the RSS feed. If you are more a fan of the visual medium then subscribe to our YouTube channel. Please embrace the spirit of FOAMed and spread the word.
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Top Tips for Paediatric Oncology Lines

Cite this article as:
Ana Waddington. Top Tips for Paediatric Oncology Lines, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.25732

Are you involved in the care of paediatric hickmans, port or picc lines  in paediatric patients? Lines, particularly those for oncology patients can sometimes leave nursing and medical staff all tangled up. Thanks to the Royal London Hospital Paediatric Oncology team, Ana Waddington and Amanda Ullman we are happy to share some handy top tips to improve line care:

    1. Use aseptic non touch technique (ANTT) when accessing Oncology patients Central venous lines 
    2. Clamping sequence is important, to prevent back-flow of blood up the device. But, the sequence (including positive vs neutral pressure) depends on the needleless connector that you use. Always check the manufacturer’s recommendations.
    3. Securing your line:  Always have at least one securement device (e.g., sutures, clasp, reinforced dressing) to keep the central line in the correct place – and two is even better
    4. Flushing: Flushing the central line with 0.9% sodium chloride after administration of viscous fluids is vital to prevent occlusion. 
    5. When accessing a totally implanted device (e.g., port-a-cathTM):
      • Consider local anaesthetic prior to insertion (e.g., LMX, Ametop, Emla)
      • Pinch the edges of the port- a cath to secure the location to insert your needle
      • Insert at 90 angle until you feel the needle hit the back
      • Don’t force it- you may cause some injury to the port chamber
      • Try repositioning yourself and the patient to an angle that feels more comfortable
      • If under the armpit, try lifting the patient’s arm to stretch the skin
      • Try not to go where there is bruising, adjust the skin
    6. There are 2 types of occlusion – Withdrawal Occlusion and Total Occlusion
        • Withdrawal Occlusion – flush gently with 0.9% Sodium chloride, get patient to look up and away from their line as they maybe causing an internal kink, change their position, if unsuccessful then can use Urokinase/Alteplase
        • Total Occlusion – Change bionector, take dressing down to check for external kinks, get patient to look up and away from their line as they maybe causing an internal kink, change their position, if unsuccessful then can use Urokinase/Alteplase
    7. No matter what the presentation (e.g., injection vs aspirate occlusion) always think through the possible causes, while problem-solving:
      • Consider mechanical occlusion: e.g., do you have malfunctioning needleless connectors? Are there external kinks? Plus [really importantly] is the tip position central? 
      • Consider infusate occlusion: i.e. have you just administered medications that may have precipitated? If so, talk to your pharmacist about how to dissolve.
      • Then think about thrombotic occlusion, and consider administering thrombolytic agents, like urokinase. If this doesn’t work, consider imaging e.g., lineogram
    8. Do not use prefilled syringes to flush off a PICC, as these are luer lock not luer slip syringes and they cause the PICCs to block
    9. Do not put heparin into a PICC line, they are to be flushed with 0.9% Sodium Chloride
    10.  If you run into trouble and are not sure what to do- make sure that you seek help with senior staff of your team, check your hospital policy/guidelines and the manufacturer instructions to solve the problem together.

For your convenience, the top tips are summarised in an A4 poster format (infographic by Grace Leo):

Bubble Wrap Plus – September 2020

Cite this article as:
Anke Raaijmakers. Bubble Wrap Plus – September 2020, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.29169

Here is a new Bubble Wrap Plus, our monthly paediatric Journal Club List provided by Professor Jaan Toelen & his team of the University Hospitals in Leuven (Belgium). This comprehensive list of ‘articles to read’ comes from 34 journals, including Pediatrics, The Journal of Pediatrics, Archives of Disease in Childhood, JAMA Pediatrics, Journal of Paediatrics and Child Health, NEJM, and many more.

This month’s list features answers to intriguing questions such as: ‘What is the effect of longer resuscitations at birth on neurodevelopment?’, ‘Is it necessary to evaluate urinary tract infection in children with lower respiratory tract infection?’, ‘What is the effect of social distancing on ‘regular’ URTIs?’, ‘Does maternal pertussis vaccination interfere with neonatal vaccination?’ and ‘Is migraine a common cause for nystagmus in the emergency department?’.

1.Reviews and opinion articles

Thinking fast and slow in the evaluation of injury plausibility in child protection.

Skellern C J Paediatr Child Health. 2020 Aug 11.

The Suffering Child: Claims of Suffering in Seminal Cases and What To Do About Them.

Friedrich AB. Pediatrics. 2020 Aug;146(Suppl 1):S66-S69.

Childhood Sexual Abuse: A Call to Action in Pediatric Primary Care.

Ghastine L, et al . Pediatrics. 2020 Aug 4:e20193327.

Kawasaki disease fact check: Myths, misconceptions and mysteries.

Butters C, et al. J Paediatr Child Health. 2020 Aug 8.

Follow the complex bread crumbs: A review of autoinflammation for the general paediatrician.

Tsoukas P, et al. Paediatr Child Health. 2020 Aug;25(5):279-285.

Plant-Based Milks: A Possible Therapeutic Tool if Correctly Labeled and Prescribed.

Mennini M, et al . J Pediatr Gastroenterol Nutr. 2020 Jul 30.

The Liver in Sickle Cell Disease.

Lacaille F, et al . J Pediatr Gastroenterol Nutr. 2020 Jul 30.

Management of Post-hemorrhagic Ventricular Dilatation in the Preterm Infant.

El-Dib M, et al . J Pediatr. 2020 Jul 30:S0022-3476(20)30978-1.

Update on the COVID-19-associated inflammatory syndrome in children and adolescents; paediatric inflammatory multisystem syndrome-temporally associated with SARS-CoV-2.

Singh-Grewal D, et al . J Paediatr Child Health. 2020 Jul 31.

2. Original clinical studies

Early Feeding in Acute Pancreatitis in Children: A Randomized Controlled Trial.

Ledder O, et al. Pediatrics. 2020 Aug 12:e20201149.

The Safety of Early Enteral Feeding in Children With Acute Pancreatitis.

Hamilton-Shield J, et al. Pediatrics. 2020 Aug 12:e2020007211.

Duration of Resuscitation at Birth, Mortality, and Neurodevelopment: A Systematic Review.

Foglia EE, et al. Pediatrics. 2020 Aug 12:e20201449.

Is it necessary to evaluate urinary tract infection in children with lower respiratory tract infection?

Kim JM, et al. J Paediatr Child Health. 2020 Aug 8.

Use of oximetry to screen for paediatric obstructive sleep apnoea: is one night enough and is 6 hours too much?

Galway NC, et al. Arch Dis Child. 2020 Aug 11:archdischild-2019-318559.

Pediatric SARS-CoV-2: Clinical Presentation, Infectivity, and Immune Responses.

Yonker LM, et al . J Pediatr. 2020 Aug 18:S0022-3476(20)31023-4.

Effect of Social Distancing Due to the COVID-19 Pandemic on the Incidence of Viral Respiratory Tract Infections in Children in Finland During Early 2020.

Kuitunen I, et al. Pediatr Infect Dis J. 2020 Jul 28.

Masked paediatricians during the COVID-19 pandemic and communication with children.

Shack AR, et al. J Paediatr Child Health. 2020 Aug 8.

Role of children in household transmission of COVID-19.

Kim J, et al. Arch Dis Child. 2020 Aug 7:archdischild-2020-319910.

Prevalence of SARS-CoV-2 Infection in Children Without Symptoms of Coronavirus Disease 2019.

Sola AM, et al . JAMA Pediatr. 2020 Aug 25.

Increased incidence of complicated appendicitis during the COVID-19 pandemic.

Lee-Archer P, et al . J Paediatr Child Health. 2020 Aug;56(8):1313-1314.

Is Nasopharyngeal Swab Comparable With Nasopharyngeal Aspirate to Detect SARS-CoV-2 in Children?

Capecchi E, et al . Pediatr Infect Dis J. 2020 Jul 21.

Community-Based Epidemiology of Hospitalized Acute Kidney Injury.

Parikh RV, et al. Pediatrics. 2020 Aug 11:e20192821.

Endocarditis prophylaxis in daily practice of pediatricians and dentists in Flanders.

De Wolf D, et al. Eur J Pediatr. 2020 Aug 11.

A 10-year retrospective survey of acute childhood osteomyelitis in Stockholm, Sweden.

von Heideken J, et al. J Paediatr Child Health. 2020 Aug 11.

Virtual reality for intravenous placement in the emergency department-a RCT

Goldman RD, et al. Eur J Pediatr. 2020 Aug 10.

Bedside Airway Ultrasound in the Evaluation of Neonatal Stridor.

Oulego-Erroz I, et al. J Pediatr. 2020 Aug 6:S0022-3476(20)30993-8.

Normal fecal calprotectin levels in healthy children are higher than in adults and decrease with age.

Velasco Rodríguez-Belvís M, et al. Paediatr Child Health. 2020 Aug;25(5):286-292.

Can Use of Cerebral Oxygenation Predict Developmental Outcomes in Preterm Infants With NEC?

Horne RSC.Pediatrics. 2020 Aug 26:e2020014407.

Cerebral Oxygenation in Preterm Infants With Necrotizing Enterocolitis.

Howarth C, et al .Pediatrics. 2020 Aug 26:e20200337.

Junior doctor perceptions of education and feedback on ward rounds.

Modak MB, et al . J Paediatr Child Health. 2020 Aug 26.

Treatment of Postural Orthostatic Tachycardia Syndrome With Medication: A Systematic Review.

Hasan B, et al . J Child Neurol. 2020 Aug 24:883073820948679.

Synchronized Inflations Generate Greater Gravity Dependent Lung Ventilation in Neonates.

Dowse G, et al . J Pediatr. 2020 Aug 19:S0022-3476(20)31029-5.

Maternal Stress During Pregnancy Predicts Infant Infectious and Non-infectious Illness.

Bush NR, et al . J Pediatr. 2020 Aug 19:S0022-3476(20)31027-1.

A Randomized Trial of Closed-Loop Control in Children with Type 1 Diabetes.

Breton MD, et al . N Engl J Med. 2020 Aug 27;383(9):836-845.

Effect of Vitamin D3 Supplementation on Severe Asthma Exacerbations in Children With Asthma and Low Vitamin D Levels: The VDKA Randomized Clinical Trial.

Forno E, et al . JAMA. 2020 Aug 25;324(8):752-760.

Interference With Pertussis Vaccination in Infants After Maternal Pertussis Vaccination.

Abu-Raya B, et al . Pediatrics. 2020 Aug 4:e20193579.

Management of pain in newborn circumcision: a systematic review.

Rossi S, et al . Eur J Pediatr. 2020 Aug 3.

Extreme prematurity, growth and neurodevelopment at 8 years: a cohort study.

Hickey L, et al . Arch Dis Child. 2020 Aug 3:archdischild-2019-318139.

Genetic Susceptibility to Life-threatening Respiratory Syncytial Virus Infection in Previously Healthy Infants.

López EL, et al . Pediatr Infect Dis J. 2020 Jul 17.

Outcomes Following Post-Hemorrhagic Ventricular Dilatation among Extremely Low Gestational Age Infants.

Shankaran S, et al . J Pediatr. 2020 Jul 30:S0022-3476(20)30979-3.

Skin-to-skin care alters regional ventilation in stable neonates.

Schinckel NF, et al . Arch Dis Child Fetal Neonatal Ed. 2020 Jul 30:fetalneonatal-2020-319136.

School-age outcomes following intraventricular haemorrhage in infants born extremely preterm.

Hollebrandse NL, et al . Arch Dis Child Fetal Neonatal Ed. 2020 Jul 30:fetalneonatal-2020-318989.

Characteristics of Acute Nystagmus in the Pediatric Emergency Department.

Garone G, et al . Pediatrics. 2020 Aug;146(2):e20200484.

3. Guidelines and Best Evidence

Automated oxygen control in preterm infants, how does it work and what to expect: a narrative review.

Salverda HH, et al . Arch Dis Child Fetal Neonatal Ed. 2020 Jul 30:fetalneonatal-2020-318918.

Diagnosis, Evaluation, and Treatment of Attention-Deficit/Hyperactivity Disorder.

Loe IM, et al. JAMA Pediatr. 2020 Aug 10.

Clinical Management of Staphylococcus aureus Bacteremia in Neonates, Children, and Adolescents.

McMullan BJ, et al. Pediatrics. 2020 Aug 5:e20200134.

Universal screening of high-risk neonates, parents, and staff at a neonatal intensive care unit during the SARS-CoV-2 pandemic.

Cavicchiolo ME, et al. Eur J Pediatr. 2020 Aug 7:1-7.

Fluid management during diabetic ketoacidosis in children: guidelines, consensus, recommendations and clinical judgement.

Tasker RC. Arch Dis Child. 2020 Aug 26:archdischild-2020-320164.

Clinical Prediction Rule for Distinguishing Bacterial From Aseptic Meningitis.

Mintegi S, et al . Pediatrics. 2020 Aug 25:e20201126.

Reducing Antibiotic Prescribing in Primary Care for Respiratory Illness.

Kronman MP, et al . Pediatrics. 2020 Aug 3:e20200038.

Reopening schools during the COVID-19 pandemic: governments must balance the uncertainty and risks of reopening schools against the clear harms associated with prolonged closure.

Viner RM, et al . Arch Dis Child. 2020 Aug 3:archdischild-2020-319963.

North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Position Paper: Plant-based Milks.

Merritt RJ, et al . J Pediatr Gastroenterol Nutr. 2020 Aug;71(2):276-281.

Airborne Transmission of SARS-CoV-2: Theoretical Considerations and Available Evidence.

Klompas M, et al . JAMA. 2020 Aug 4;324(5):441-442.

4. Case Reports

A 16-Year-Old Boy With Cough and Fever in the Era of COVID-19.

Anderson KR, et al. Pediatrics. 2020 Aug 12:e2020008235.

An Infant Presenting with Large, Asymmetric Tongue.

Golomb MR, et al. J Pediatr. 2020 Aug 7:S0022-3476(20)30992-6.

Tracheal Buckling in a Young Child.

Miyamoto M, et al. J Pediatr. 2020 Aug 6:S0022-3476(20)30991-4.

Methaemoglobinaemia in two exclusively breastfed infants with food protein-induced enterocolitis syndrome.

Geljic A, et al. J Paediatr Child Health. 2020 Aug 7.

Recurrent pneumothorax in a child.

Rajvanshi N, et al . J Paediatr Child Health. 2020 Aug 5.

Sequential Retinal Hemorrhages in an Asymptomatic Child.

Ho DK, et al . J Pediatr. 2020 Jul 30:S0022-3476(20)30980-X.

Agranulocytosis and lymphopenia in neonate: A neonatal emergency.

Rustogi D, et al. J Paediatr Child Health. 2020 Aug 7.

If we have missed out on something useful or you think other articles are absolutely worth sharing, please add them in the comments!

Sickle cell disease

Cite this article as:
Rowenne Smith. Sickle cell disease, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.27598

Abigail is a 10 month-old female presenting to the Emergency Department with a history of profound lethargy, pallor and a mildly distended abdomen over the last few hours. She has no past medical history, her immunisations are up to date, and she has no known allergies. Her parents report that she is usually well, however they have noticed recent swelling of her hands and feet over the last month. Her parents are originally from Nigeria. In the Emergency Department she is pale and flat. 

She is tachycardic with a heart rate of 180bpm and afebrile. On examination she has a prolonged capillary refill time of 5 seconds and cool peripheries. She has a soft systolic murmur. Her spleen measures 7cm below the costal margin and she squirms on abdominal palpation. There is no history or evidence of trauma. 

Intravenous (IV) access is obtained and bloods are sent including a venous blood gas, blood culture, FBE, blood group and cross match, LFT and UEC. 

Abigail is given a 10ml/kg fluid bolus of 0.9% sodium chloride and commenced on broad-spectrum antibiotics. After a second 10ml/kg bolus her capillary refill time and heart rate improve but she remains very lethargic. 

You are notified by pathology that Abigail’s formal haemoglobin is 64 g/L and she is thrombocytopaenic with a platelet count of 80 x 109/L. The white cell count is within normal limits. 

You send for an urgent blood transfusion and arrange a PICU review.

The treating team is contacted by the haematologist who has reviewed her blood film and noted the presence of target cells, Howell-Jolly bodies and sickle cells. 

What is the diagnosis? Abigail has presented in hypovolaemic shock secondary to splenic sequestration as a first presentation of sickle cell disease. 

What is sickle cell disease?

Sickle cell disease (SCD) is a genetic disorder of haemoglobin synthesis.

Haemoglobin is a tetramer comprised of four polypeptide globin chains, each containing a haem molecule (which reversibly binds oxygen). Beyond infancy, adult haemoglobin (HbA) replaces foetal haemoglobin (HbF) as the predominant haemoglobin molecule. HbA consists of two alpha and two beta globin chains. 

SCD is caused by a point mutation in the beta globin gene resulting in a structurally abnormal haemoglobin molecule, HbS. 

The primary event in sickle cell pathology is polymerisation of HbS, distorting the red cell shape and leading to the characteristic sickle appearance.  Polymerisation can occur in the setting of deoxygenation, acidosis, pyrexia and dehydration. Recurrent episodes of sickling cause red blood cell (RBC) membrane damage and an irreversibly sickled cell.

Sickled RBCs adhere to the vascular endothelium and circulating RBCs causing occlusion of the microvascular circulation (vaso-occlusion). Sickled RBCs also undergo haemolysis, with an average RBC lifespan of only 17 days.

The physiological changes in RBCs result in a multisystem disease with the following key features:

  • Chronic haemolytic anemia
  • Painful vaso-occlusive episodes
  • Multi-organ damage from micro-infarcts (including cardiac, skeletal, splenic and central nervous system).

Inheritance and incidence

Sickle cell anaemia is inherited in an autosomal recessive pattern.

It is one of the most common, severe monogenic disorders worldwide. The prevalence of the disease is high among individuals of sub-Saharan African, Indian, Saudi Arabian and Mediterranean descent. 

It is estimated that 312 000 neonates are born with sickle cell anaemia globally each year, over 75% of whom are born in sub-Saharan Africa.

Terminology

SCD refers to a group of disorders characterised by the presence of at least one HbS allele in addition to a second beta globin gene mutation. 

In sickle cell anaemia, individuals are homozygous for HbS (HbSS). This is the most frequent and severe form of the disease. Other variants of SCD include sickle β thalassaemia (HbSβ0 or HbSβ+ thalassaemia) and haemoglobin SC disease (HbSC)

Individuals with sickle cell trait are benign carriers for the condition, inheriting HbS and a normal beta globin gene (HbAS). Sickle cell trait confers a survival advantage in malaria endemic areas. 

Diagnosis

Universal newborn screening for SCD has been implemented in the United States and United Kingdom. SCD is not part of the newborn screening program in Australia.

SCD can be diagnosed through the identification of haemoglobin variants using haemoglobin electrophoresis, high-performance liquid chromatography (HPLC) or isoelectric focusing. 


Clinical manifestations 

Symptom onset usually occurs within the first year of life, often at around 5 months. The delay in clinical signs and symptoms is due to the higher levels of HbF in infancy preventing the polymerisation of HbS.

Clinical manifestations include:

Anaemia

  • Patients have a chronic, compensated haemolytic anaemia.
  • Major causes of an acute drop in haemoglobin include splenic sequestration and aplastic crisis. 
  • Aplastic crisis is caused by a transient arrest in erythropoiesis. This is typically caused by infection, commonly human parvovirus B19.

Vaso-occlusive pain episodes

  • This is the cardinal feature of SCD and accounts for the majority of hospital admissions.
  • Acute pain occurs due to ischaemic tissue injury secondary to vaso-occlusion of sickled cells.
  • The majority of episodes have no identifiable cause, however common triggers include infection, fever, acidosis, hypoxia, dehydration and exposure to temperature extremes. 
  • Common sites of pain include the chest, abdomen, back and extremities. Dactylitis is a common presentation in infants and toddlers, with back and abdominal pain more common in older children.
  • Management of vaso-occlusive episodes involves early and aggressive pain relief.

Splenic sequestration 

  • Splenic sequestration occurs when large quantities of sickled RBCs pool within the spleen. This is a potentially life threatening complication of SCD, with a risk of hypovolaemic shock.
  • Splenic sequestration is characterised by the sudden enlargement of the spleen, an acute drop in haemoglobin (>20 g/L), thrombocytopaenia and an increase in reticulocytes.
  • It typically occurs between the ages of 6 months and 2 years.
  • Management includes: 
    • Restoration of circulating blood volume with a blood transfusion. This increases the haemoglobin level directly and promotes the release of trapped RBCs by the spleen. 
      • Always discuss transfusion targets with the on-call haematologist, as autotransfusion will occur if haemoglobin is increased excessively or too quickly thereby increasing the risk of hyperviscosity syndrome. 
    • Active fluid resuscitation for hypovolaemia while awaiting a blood transfusion.

Infection

  • Functional hyposplenism occurs early in life due to splenic infarction.
  • Patients are at an increased risk of invasive bacterial infections, particularly by encapsulated organisms (Streptococcus pneumoniae, Haemophilus influenzae type B and Neisseria meningitidis).
  • Children with SCD presenting with a febrile illness require prompt assessment and empiric IV antibiotics.
  • Prevention strategies include: 
    • Prophylactic penicillin for all young children, ideally by the age of 2-3 months. This has been shown to significantly reduce the morbidity and mortality of pneumococcal infections.
    • Vaccinations as per the functional asplenia/hyposplenia guidelines.

Acute chest syndrome (ACS)

  • This is the leading cause of mortality in patients with SCD.
  • ACS is defined as a new infiltrate on chest x-ray associated with new respiratory symptoms (chest pain, respiratory distress, hypoxia or cough) and/or fever. 
  • The majority of patients do not have a single identifiable cause. Possible aetiologies include infection, atelectasis, vaso-occlusion and fat emboli from infarcted bone marrow.
  • Management includes supplemental oxygen, IV antibiotics, exchange transfusion, analgesia, physiotherapy and early PICU involvement if hypoxia or respiratory distress.

Stroke

  • Prior to routine screening with transcranial doppler ultrasound (TCD), clinically evident strokes occurred in up to 11% of patients with SCD by the age of 20 years.
  • Silent cerebral infarcts (evidence of infarction on neuroimaging in the absence of overt neurological symptoms) occur in up to 20% of children with sickle cell anaemia.
  • Management includes: 
    • Prompt neuroimaging – MRI is the modality of choice, however if unavailable non-contrast CT should be performed (contrast increases the risk of hyperviscosity).
    • Exchange transfusion.
  • Primary prevention strategies include:
    • Regular TCD assessment starting from the age of 2 years. 
    • Prophylactic regular transfusions for children with persistently elevated TCD velocity.

Priapism

  • Priapism is an unwanted, persistent erection in the absence of sexual activity.
  • The majority of episodes occur due to impaired venous outflow from the penis causing increased pressure, preventing normal arterial circulation.
  • Prolonged episodes of priapism (>4 hours) may lead to permanent tissue damage, with a risk of erectile dysfunction.
  • The optimal treatment is unknown. 
  • Management strategies include hydration, analgesia, oxygen therapy, showering, short aerobic exercise and urination (consider catheterisation if unable to empty bladder). Ice should not be used as cold temperatures may exacerbate sickling. 
  • Priapism extending beyond 4 hours is a urological emergency and consultation with the on-call haematologist and general surgical/urology team is required.

Avascular necrosis

  • Avascular necrosis occurs at a higher rate in children with SCD.
  • It commonly affects the femoral and humeral heads.

Sickle cell disease and COVID-19


There is limited data on the relationship between SCD and COVID-19. Children with sickle cell disease, thalassaemia and rare anaemias without other risk factors do not seem to be at increased risk of having severe disease. 

Emergency department management

Examination

  • Vital signs
  • Pallor or jaundice
  • Hydration status
  • Respiratory examination
  • Spleen examination, with comparison to baseline
  • Neurological examination
  • Localising signs of infection

Investigations

  • FBE and reticulocyte count
    • Splenic sequestration: haemoglobin below baseline, thrombocytopaenia, reticulocytosis
    • Aplastic anaemia: haemoglobin below baseline, decreased reticulocyte count (<1%)
  • Blood group and cross match
  • UEC and LFT (if jaundice or dehydrated)
  • Based on assessment
    • If febrile 🡪 blood and urine culture
    • If respiratory symptoms 🡪 consider chest x-ray
    • If neurological findings 🡪 urgent neuroimaging

Acute management

  • Prompt review and early discussion with the on-call haematologist.
  • Aggressive pain management – all patients with SCD presenting with pain should initially be managed as a vaso-occlusive episode, with the exception of chest pain, which should be treated as ACS.
  • Oxygen therapy for hypoxia or respiratory distress, aiming for SaO2 >96% or for comfort.
  • Fluid management:
    • Encourage oral fluids.
    • Consider IV fluids for fluid resuscitation or maintenance fluids if unable to tolerate oral intake.
    • It is important to recognise that excessive fluid administration can increase the risk of ACS.
  • A blood transfusion may be required, however this should always be in consultation with the on-call haematologist to discuss both the type of transfusion and transfusion targets. 
    • There is a risk of hyperviscosity if the haemoglobin is increased significantly over the patient’s baseline.
  • If febrile, commence IV antibiotics with a third generation cephalosporin, in addition to atypical coverage if there is a significant respiratory component. 
  • If respiratory symptoms, suspect ACS.

Chronic management

Blood transfusions are used to treat and prevent the complications of SCD. Types of transfusions include simple, manual partial exchange and automated red cell exchange (erythrocytapheresis).  

Hydroxyurea is a myelosuppressive agent used in the management of individuals with SCD, which has been shown to reduce the vaso-occlusive complications. 

A life-long cure for SCD is only available through haematopoietic stem cell transplantation.

Prognosis

Individuals with SCD have reduced overall life expectancy. In high-income countries, the survival of individuals with SCD is improving steadily through measures such as newborn screening, early initiation of antibiotic prophylaxis, immunisations and screening for children at high risk of stroke.

This is not the case worldwide. The majority of countries where SCD is a major public health concern lack national programs and key public health interventions. As a result, sickle cell anaemia-related childhood mortality in Africa is as high as 50-90%, with less than half of affected children reaching the age of five. The World Health Organization (WHO) estimates that 70% of sickle cell anaemia deaths are preventable with simple, cost-effective interventions. 

Key messages

SCD is a multisystem disease characterised by haemolytic anemia, painful vaso-occlusive episodes and multi-organ damage from micro-infarcts.

Early diagnosis, simple prophylactic measures and parental education improves the morbidity and mortality of SCD.

Always discuss with the on-call hematologist prior to transfusing a sickle cell patient due to the risk of hyperviscosity. 

References

Arlet JB, de Luna G, Khimoud D, et al. Prognosis of patients with sickle cell disease and COVID-19: a French experience [published online ahead of print, 2020 Jun 18]. Lancet Haematol. 2020;S2352-3026(20)30204-0. doi:10.1016/S2352-3026(20)30204-0

Bainbridge R, Higgs DR, Maude GH, Serjeant GR. Clinical presentation of homozygous sickle cell disease. J Pediatr. 1985;106(6):881-885. doi:10.1016/s0022-3476(85)80230-4

Brousse V, Buffet P, Rees D. The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol. 2014;166(2):165-176. doi:10.1111/bjh.12950

Dick M, Rees D. Sickle Cell Disease in Childhood: Standards and Recommendations for Clinical Care (3rd edition, 2019). Available at https://www.sicklecellsociety.org/wp-content/uploads/2019/11/SCD-in-Childhood_Final-version-1.pdf [accessed 24 June 2020]

Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, Williams TN. Sickle cell disease in Africa: a neglected cause of early childhood mortality. Am J Prev Med. 2011;41(6 Suppl 4):S398-S405. doi:10.1016/j.amepre.2011.09.013

Meier ER, Miller JL. Sickle cell disease in children. Drugs. 2012;72(7):895-906. doi:10.2165/11632890-000000000-00000

Odunvbun ME, Okolo AA, Rahimy CM. Newborn screening for sickle cell disease in a Nigerian hospital. Public Health. 2008;122(10):1111-1116. doi:10.1016/j.puhe.2008.01.008

Pace BS, Goodman SR. Sickle cell disease severity: an introduction. Exp Biol Med (Maywood). 2016;241(7):677-678. doi:10.1177/1535370216641880

Piel FB, Patil AP, Howes RE, et al. Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates. Lancet. 2013;381(9861):142-151. doi:10.1016/S0140-6736(12)61229-X

Therrell BL Jr, Lloyd-Puryear MA, Eckman JR, Mann MY. Newborn screening for sickle cell diseases in the United States: A review of data spanning 2 decades. Semin Perinatol. 2015;39(3):238-251. doi:10.1053/j.semperi.2015.03.008

World Health Organization. Geneva. World Health Organization – 59th World Health Assembly resolutions; 2006. Sickle-cell anaemia. https://apps.who.int/gb/archive/pdf_files/WHA59/A59_9-en.pdf [Accessed on 30th June 2020]

Emotional Contagion: Andrew Tagg at DFTB19

Cite this article as:
Andrew Tagg. Emotional Contagion: Andrew Tagg at DFTB19, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.22095

I’ve always had an affinity for John Carter, as played by Noah Wyle, from the TV series ER. The night ER premiered on UK television happened to be my very first ED shift as a medical student at the Chelsea and Westminster Hospital.  I remember sneaking away to the lounge to watch it. My first ED experience was nothing like Carters but I was hooked.

I went to Chicago to do my elective and saw them filming in the snow in between shifts at Northwestern and when I returned to England to prepare for finals we would gather around the TV trying to make the diagnosis before the medicos on the television. We called it revising, but really it was escaping from the textbooks for a short time.

My interest wained nearer the end of the run. Carter had been through many trials, as had I, but one thing has stuck with me more than anything else. It was something passed down from David Morgenstern (William H. Macy) to Mark Greene (Anthony Edwards), and then more importantly from Greene to Carter. That is the basis for this talk. You can read the background here.

 

 

 

 

This talk was recorded live at DFTB19 in London, England. With the theme of  “The Journey” we wanted to consider the journeys our patients and their families go on, both metaphorical and literal.

If you want our podcasts delivered straight to your listening device then subscribe to our iTunes feed or check out the RSS feed. If you are more a fan of the visual medium then subscribe to our YouTube channel. Please embrace the spirit of FOAMed and spread the word.

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