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.
iTunes Button

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.

iTunes Button
 

 

Safety Netting

Cite this article as:
Carl van Heyningen. Safety Netting, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.28803

Safety netting is a cornerstone of paediatric practice. 

Children are a vulnerable group. Their condition can deteriorate and improve rapidly. Uncertainty is inherent in paediatric emergency medicine. From the child with a fever to the infant with vomiting, it is up to us to safeguard children from harm.  

Of course, we can’t admit every child to the hospital. Nor should we. The vast majority of patients in A&E return home with reassurance. Easy right? Reassure. Give advice. Send home. Simple?

You’re at the end of a busy shift and you have a train to catch. You’ve put in blood, sweat, and tears and feel happy your last patient has a simple throat infection. You advise fluids, paracetamol for symptoms of headache and neck pain and to come back if worried. The mother is anxious, you give them a leaflet on fever and rush out the door. 

Typically, our focus is on the front door of care not the exit. Discharge care is often overlooked. Even in the best of circumstances, we are under pressure to maintain patient flow especially as our emergency departments begin to become busy again. 

The next morning, coffee in hand, you walk in the department and hear the words that strike fear into the hearts of all that hear them… 

“Do you remember that child you saw yesterday? They’re being admitted to intensive care, it looks like meningitis.”

What could you have done differently? More tests? Not necessarily needed, no? More time? They had been observed and appeared well for several hours. Senior review? You’d seen them with the Consultant and agreed on the diagnosis. Then what? 

Safety netting

Why is it important? 

Acute illness remains one of the most important causes of childhood mortality in the UK. Early illness is notoriously non-specific. Take meningitis. In only half of cases, diagnosis is made at the first presentation. So what do we do? We must educate parents about uncertainty. Discuss the potential for deterioration. Explain the importance of seeking further help if necessary. We must safety net. 

What is it?

The term was first formally described in 1987. Today, it has come to mean “advice about what to do and what to look out for to empower parents and carers to seek help if the child’s condition deteriorates further or if they need more support.”  

What else should it cover? 

In addition to the above, it is critical to cover how they should seek help, what they should expect ahead (the disease course) and when to become worried.

How should it be done? 

Whilst verbal and written formats exemplify current practice, ranging from information leaflets to printed discharge letters, audiovisual and online resources are growing in abundance. Families report wanting this varied range of approaches.

Let’s consider the options. 

Face to Face – individualized, personal but highly variable and time-dependent

TOP TIP – be adaptable (don’t just simply recite the same information each time)

Social, educational, and cultural differences may all necessitate adapting your usual spiel in order to truly achieve understanding. Remember, the parents are in an unfamiliar, often noisy, and stressful environment in addition to feeling worried about their child. Expect them to be distracted a little. 

Leaflets – standardised, quality assured but not necessarily up to date and potentially bland and uninteresting. 

TOP TIP – use leaflets to re-enforce verbal information

When taken home written materials can often act as an aide memoire.  

Audio-visual – engaging and memorable with the potential to overcome literacy and language barriers if well designed, though resource intensive and expensive upfront   

Internet, social media, websites, apps – there are many innovative methods of connecting families with health information. Our responsibility is thus to navigate the clutter, signpost reliable resources, dispel myths and thus champion true evidence based materials. 

TOP TIP – keep the message simple, it can be easy to overwhelm parents with information. 

Remember, many parents may not wish to go on the internet. A few may even not have access to it. 

Why tailor the information? 

As ever, before talking we must first listen. For example, one interview based study found a mother worrying about “their child with cough dying at night through choking on phlegm.” If we do not listen to such fears how can we expect our own advice to be heard. 

Parental priorities

Parents priorities include… 

Emotional distress (addressing this)

Physical symptoms (addressing these)

Information (providing this, particularly reassurance, diagnosis and explanation)

Care (basic care, including food drink and friendliness)

Closure (finding out what’s wrong and, where possible, going home)

An awareness of these priorities can inform our conversations, helping us to better look after our patients from their perspective.

Ok, but what is the reality?

“You don’t actually know how much of that leaflet they’re gonna actually understand, take in, comprehend… going through things step by step, listening, understanding and explaining, I think is more beneficial” (Paediatric ED doctor). 

“It’s very difficult to know ‘cause often they’ll nod their heads and say “yes I understand everything you say” and walk off and they might have no idea what we’ve just said”  (ED staff nurse). 

“If you’ve got a sick child at home and they’re moaning at you, you haven’t got the time to go on the internet… you’ve got a child hanging off your leg going, “Mummy I feel poorly, mummy I want this, mummy I want that”” (Mother).

“My doctor did give like an information leaflet… and I did read through it, because when you’ve got a sheet at least you can find time to do that” (Mother).

So how can we do it best?

After reviewing the literature and FOAMed (see further resources) here are my top tips for giving the very best safety netting advice, enjoy! 

  • Sit down – it has been shown to increase the perception of empathy 
  • Verbalize back concerns – be explicit important conditions have been excluded 
  • Explain things – share reasoning, show your process 
  • Highlight red flags – signs that necessitate reattendance
  • Be specific – ‘If x happens, do y’
  • Reinforce – provide written leaflets
  • Avoid criticism, foster understanding – put yourself in their shoes 
  • Document advice – yes, write down what you said 

And as with all good communication, ensure a quiet, private area and avoid using jargon. 

Finally, directly ask if parents understand and are happy. Don’t assume they are. 

Here is one good example to get you started… 

“…your little guy is likely to continue to have vomiting and diarrhoea. If he remains well in himself, is drinking the amount of fluid we have discussed and is having wet nappies then he is unlikely to become dehydrated. If, however, he becomes drowsy, develops a fever or fails to stay hydrated please call this number and come back to us.” 

We must stop thinking of reattendance as a failure – patients do get worse and some need to return. 

Good quality safety netting means both you and your patients can get a better nights sleep. 

References

Gill P, Goldacre M, Mannt D, Heneghan C, Thomson A, Seagroatt V and Harnden A (2013) ‘Increase in emergency admissions to hospital for children aged under 15 in England, 1999–2010: national database analysis’, Archives of Disease in Childhood 98, 328–34.

Wolfe I, Cass H, Thompson MJ, Craft A, Peile E, Wiegersma PA, Janson S, Chambers T, McKee M: Improving child health services in the UK: insights from Europe and their implications for the NHS reforms. Bmj 2011, 342:d1277.2. 

Thompson MJ, Ninis N, Perera R, Mayon-White R, Phillips C, Bailey L, Harnden A, Mant D, Levin M: Clinical recognition of meningococcal disease in children and adolescents. Lancet 2006, 367(9508):397–403.3. 

Neighbour R. The inner consultation. Lancaster: MTP Press, 1987.

NICE guideline [NG143], Fever in under 5s: assessment and initial management, November 2019

Available at https://www.nice.org.uk/Guidance/Ng143/evidence

Almond S, Mant D, Thompson M: Diagnostic safety-netting. The British journal of general practice: the journal of the Royal College of General Practitioners 2009, 59(568):872–874

Jones CH, Neill S, Lakhanpaul M, et al. Information needs of parents for acute childhood illness: determining what, how, where and when of safety netting using a qualitative exploration with parents and clinicians. BMJ Open 2014;4:e003874.

Neill SJ, Jones CH, Lakhanpaul M, et al. Parent’s information seeking in acute childhood illness: what helps and what hinders decision making? Health Expect 2015;18:3044–56. 

Austin PE, Matlack R, Dunn KA, et al. Discharge instructions: do illustrations help our patients understand them? Ann Emerg Med 1995;25:317–20.

Scullard P, Peacock C, Davies P. Googling children’s health: reliability of medical advice on the internet. Arch Dis Child 2010;95:580–2.

Mackert M, Kahlor L, Tyler D, et al. Designing e-health interventions for low-health-literate culturally diverse parents: addressing the obesity epidemic. Telemed J E Health 2009;15:672–7.

Knight K, van Leeuwen DM, Roland D, et al. YouTube: are parent-uploaded videos of their unwell children a useful source of medical information for other parents? Arch Dis Child 2017;102:910–4.

CS Cornford, M Morgan, L Risdale, Why do Mothers Consult when their Children Cough?, Family Practice, Volume 10, Issue 2, July 1993, Pages 193–196

Body R, Kaide E, Kendal S, et al. Not all suffering is pain: sources of patients’ suffering in the emergency department call for improvements in communication from practitioners, Emergency Medicine Journal 2015;32:15-20.

(15) Jones, C.H., Neill, S., Lakhanpaul, M. et al. The safety netting behaviour of first contact clinicians: a qualitative study. BMC Fam Pract 14, 140 (2013)

Jones, C.H.D., Neill, S., Lakhanpaul, M., Roland, D., Singlehurst-Mooney, H. and Thompson, M., (2014) Information needs of parents for acute childhood illness: determining ‘what, how, where and when’ of safety netting using a qualitative exploration with parents and clinicians. BMJ Open 4 (1). 

Further resources

RCPCH (2015) Facing the Future: Standards for acute general paediatric services. RCPCH.

RCPCH Safe System Framework, resources accessed 19th November 2019,  https://www.rcpch.ac.uk/resources/safe-system-framework-children-risk-deterioration

Dr. Natalie May, MBChB, MPHe, MSc, PGCert Medical Education, FRCEM, FACEM, #CommunicatED 1: Discharge & Safety Netting in ED, available at https://www.stemlynsblog.org/communicated-discharge-safety-netting/

Bruera, Eduardo & Palmer, J Lynn & Pace, Ellen & Zhang, Karen & Willey, Jie & Strasser, Florian & Bennett, Michael. (2007). A randomized, controlled trial of physician posture when breaking bad news to cancer patients. Palliative medicine. 21. 501-5.

Sarah Jarvis, Medico-legal adviser

BSc MBBS MRCGP, Playing it safe – safety netting advice, available at https://mdujournal.themdu.com/issue-archive/issue-4/playing-it-safe—safety-netting-advice

Damian Roland, BMedSci (Hons) MBBS MRCPCH, PhD, TIGHTEN UP YOUR SAFETY NET #WILTW, available at 

https://rolobotrambles.com/tightenyoursafetynet/

Safety netting – a guide for professionals and parents of sick kids from GP Paedtips

Shame. How it affects patients and their relationships with health care professionals. https://abetternhs.wordpress.com/2012/11/16/shame/