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Disclaimer. I have no actual or potential conflict of interest to declare. Photographs, images, charts and information were selected from The Hospital for Sick Children teaching file, my personal collection or downloaded from the internet.Dr. D. A. Jarvis. Learning Objectives. In this session
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1. 1st Paediatric Emergency ConferenceKuwait October 2011 Sickle Cell Disease
Optimising Paediatric Emergency Care
Dr. D. A Jarvis
2. Disclaimer I have no actual or potential conflict of interest to declare.
Photographs, images, charts and information were selected from The Hospital for Sick Children teaching file, my personal collection or downloaded from the internet.
Dr. D. A. Jarvis
3. Learning Objectives In this session participants will:
Review the common presentations of Sickle Cell Disease and emergency management
Learn some of the new emerging information on approaches to management
Be stimulated to review the care Sickle Cell patients receive in their practice settings
4. What is Sickle Cell Disease? 1910 James Herrick described RBCs distorted by polymerized, deoxygenated hemoglobin as sickle shaped
1922 Mason named condition sickle cell anemia
5. Sickle Cell Genetics
6. How do patients present? Newborn screening program
Recurrent crises:
vaso occlusive (pain)
bacteremia/septicaemia
acute chest syndrome
splenic/hepatic sequestration stroke aplastic
7. Pathophysiology and Presentations recurrent ischemia due to vaso-occlusion
acute and chronic inflammation
haemolysis associated vasculopathy
Clinical presentations:
gallstones,retinopathy, priapism pulmonary hypertension, avascular bone necrosis
Individual clinical severity based on interaction of genetic/environmental factors and HbF levels
8. Vaso-Occlusive (Pain) Crisis: cause: ischemic tissue injury results from obstructed blood flow by sickled RBCs
precipitants: infection, fever, acidosis, hypoxia, dehydration, sleep apnea, extremes of heat and cold
clinical: bone, back, abdomen
infants dactylitis (hand/foot syndrome)
9. Vaso-Occlusive (Pain) Crisis: Management:
accurate ongoing pain assessments
maintain hydration 1˝ x maintenance
10 ml/kg bolus if morphine RX
adequate pain control
ą antipyretics and antihistamines
rule out other pathologies
discharge planning:
to ensure continued symptom relief and appropriate follow up health care
10. Pain Scales
11. Pain Scales
12. RT 8 Year Old Boy known sickler HbSS
presented with painful, swollen right upper arm, unable to feed himself
pain on and off for one week treated at home with acetaminophen and ibuprofen
family denies fever concerned arm is swelling and skin feels warm
On examination: pain 10/10 Temp 37.8°Celsius
Emergency staff called Child Protection Services when x-ray showed new bone formation
Was this appropriate? What is the diagnosis?
13. RT 8 Year Old Boy family contacted Sickle Clinic Nurse for help
On further investigation:
multifocal osteomyelitis
Salmonella species
Admitted to hospital:
intravenous antibiotics and surgical drainage
Further history: diarrhea one month earlier on visit to Caribbean, denied rectal bleeding or fever
There may be more than 1 pathological process present!
SickKids Toronto suggests any axillary temperature > 37.5°C be investigated
14. Bacteremia / Septicemia sickle disorders result in susceptibility to overwhelming infection with encapsulated organisms (pneumococcus / salmonella)
prophylactic antibiotics save lives
vaccinations essential
prompt evaluation with ANY rise in temperature, appropriate cultures and broad spectrum antibiotics within 30 minutes of arrival in Emergency Department
admission to hospital in most cases
15. JJ 6 Month Old Boy newborn Sickle Screen positive, confirmed at four months of age
family doctor referred baby to tertiary centre
before first visit developed a cold and temperature of 38°C
family treated with acetaminophen
found lifeless in crib
post mortem diagnosis Pneumococcal Sepsis
Could this have been prevented?
How are newly diagnosed sicklers managed in your Emergency?
16. Acute Chest Crisis: causes: infectious bacteria, viruses
non-infectious pulmonary infarction
hypoventilation, fat embolus, pulmonary oedema
clinical: young children fever + cough
adults severe pain, dysnoea, chills
chest crises cause 25% SCD fatalities
may result in restrictive chronic lung disease
17. Chest Syndrome Management oxygen saturation and/or oxygen Rx
blood culture + CBC diff + reticulocytes
chemistry + X match CXR + arterial gas
iv N/S 1-1 ˝ maintenance, bolus only if in shock
DO NOT OVER HYDRATE
iv antibiotics
measure pain + morphine iv
review old records and consult expert may need an exchange transfusion must be in ICU
18. Acute Sequestration
Clinical:
sudden increase in spleen size, decrease in haemoglobin and increase in reticulocytes
child may present with pallor, decreased activity or irritability
after attack, spleen decreases in size
In Jamaican cohort: accounted for 24% deaths in first ten years of study
Sergeant
19. Acute Splenic Sequestration Management:
parental detection reduces mortality
after 2nd attack, further events occur at shorter intervals, each associated with mortality
prophylactic splenectomy generally indicated after 2nd attack
no demonstrated disadvantages of splenectomy in terms of post-splenectomy infections or deaths
beware of over-transfusion
20. Cerebrovascular Accidents and SCD Clinical:
11% risk of stroke in HbSS patients by 20 years age 16% have silent strokes
presentation may be subtle and transient:
visual field defects / aphasia
acute changes in behaviour
motor dysfunction
First indication of stroke(s) may be school or behavioural difficulties
Hulbert et al Blood 2011 .... Progressive cerebral infarcts occurred in 45% (18 of 40 children) while receiving chronic blood transfusion therapy....
21. Cerebrovascular Accidents and SCD Management:
CT study early may be normal
MRI investigation of choice
always rule out meningitis and other causes of altered mental status
exchange transfusion
Note: educational implications and need for multidisciplinary follow up
22. AC 6 Year Old Girl A camp counsellor at Summer Camp notices 6 year old AC is watching activities today
AC states she is fine, just dont feel like playing today
How should the counsellor proceed?
What do you think is happening?
23. Sickle Cell Crisis Challenges wide spectrum of presentations
stoic / calm patients versus distressed patients
low grade fever at home, well looking
patient with normal temperature in emergency
young patients reported to be quieter than usual or not usual selves
Note: avoid underestimations of pain severity
early signs of sepsis, hypersplenism and aplastic crises may be subtle
multiple pathologies may be present
24. New emerging information can clinical severity be predicted?
do all febrile sicklers need admission?
how much fluid is enough?
what is that heart murmur?
how long do sicklers live?
nitric oxide inhalation during pain crisis
25. Can clinical Severity be Predicted? Vanden Tweel Am J Hematol 2010; 85: 746-51
Validated a pediatric severity index for sickle cell patients:
Bone necrosis
Cerebral infarcts or vasculopathy
Hepatic / Splenic sequestration
Pneumococcal sepsis and / or meningitis
Priapism
Acute chest syndrome / Painful crises
Lab values: Hb = 6.6 g/dl HbF = 3.0%
LDH 700 U/I WBC = 15.2
26. Do all febrile sicklers need admission? Rogovik et al Am J Emerg Med 2010; 28: 511-4
retrospective chart review 692 emergency visits during 2 year period Toronto SickKids
77 % febrile Sickle Cell children admitted
1.3% positive blood cultures no S. pneumoniae
most children had received 7 valent pneumoccal conjugate vaccine
Ramakrishnan M et al Lancet Inf Dis 2010
Increased risk of invasive bacterial infections in Africa....
Suggest: CAUTION continue to treat and admit febrile sickle cell patients until low risk criteria for discharge are validated
27. How much fluid is enough? Miller Pediatr Blood Cancer 2010; 54;265-8
1928 Dr. Hugh Josephs first noted that urine concentration defect common in sickle cell anaemia
defect may be reversible with transfusions
clinically nocturia, enuresis and tendency to dehydration seen
BABY HUG ongoing double blind, placebo controlled trial to evaluate efficacy of hydroxyurea in young sickle children to prevent end organ damage
Results: infants 7.5 to 17.9 months able to concentrate urine after fluid deprivation before enrolment
retesting of same children planned in 2 years
Note: avoid over hydration of young infants
28. What is that murmur? chronic anaemia
fluid overload
pulmonary hypertension: tricuspid regurgitation
pathology not related to sickle cell anaemia
Johnson 2010 Blood
Left ventricular hypertrophy and diastolic dysfunction... are related to asleep and waking oxygen saturation
Liem 2010 Am J Hematology
Reproducibility of tricuspid jet velocity measurements
Arslankoylu 2010 J Tropical Pediatrics
Assessment of cardiac functions... doppler myocardial performance index
30. References Nitric Oxide for Inhalation in the Acute Treatment of Sickle Cell Pain Crisis: a randomized trial
Gladwin MT, Kate GJ, Weiner D et al
JAMA 2011; 305 (9): 893-902
Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG)
Wang WC et al, BABY HUG investigators
The Lancet 2011; 377: 1663-72
Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with Sickle Cell Disease
Hulbert ML, McKinstry RC, Lacey JL et al
Blood 2011; 117: 772-79
The Pediatric Hydroxyurea Phase III Clinical Trial (BABY HUG) Challenges of Study Design
Thompson BW, Miller ST, Rogers ZR, Rees RC et al
Pediatr Blood Cancer 2010; 54: 250-55
31. References Urine Concentration Ability in Infants with Sickle Cell Disease: Baseline Data from the Phase III Trial of Hydroxyurea (BABY HUG)
Miller ST, Wang WC, Iyer R et al
Pediatr Blood Cancer 2010; 54: 265-68
Outcome of children with sickle cell disease admitted to intensive care a single institution experience
Bartram JL, Thein SL, Gardner K et al
Br J Haematol 2010; 150: 614-7
Left ventricular hypertrophy and diastolic dysfunction in children with sickle cell disease are related to asleep and waking oxygen desaturation
Johnson MC, Kirkham FJ, Redline S et al
Blood 2010; 116(1): 16-21
Assessment of cardiac functions in Sickle Cell Anemia with doppler myocardial performance index
Arslankoylu AE, Hallioglu O, Yilgor E, Duzovali O
J Trop Pediatr 2010; 56(3):195-7
32. References Improved survival of children and adolescents with sickle cell disease
Quinn CT, Rogers RR, McCavit TL, Buchanan GR
Blood 2010; 115: 3447-52
Development and validation of a pediatric severity index for sickle cell patients
van Den Tweel XW, van der Lee JH, Heijboer H, Peters M, Fijnvandraat K
Am J Hematol 2010; 85: 746-51
The risks and benefits of long term use of hydroxyurea in sickle cell anaemia: A 17.5 year follow up
Steinberg MH, McCarthy WF, Castro O et al; Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia and MSH Patients' Follow-Up
Am J Hematal 2010; 85: 403-8
Bacterial blood cultures in children with sickle cell disease
Rogovik AL, Friedman JN, Persaud J, Goldman RD
Am J Emerg Med 2010; 28: 511-14
33. References Increased risk of invasive bacterial infections in African people with sickle-cell disease: a systemic review and meta-analysis
Ramakrishnan M, Moisi JC, Klugman KP et al
Lancet Inf Dis 2010; 10:329-37
Pulmonary Complications of Sickle Cell Disease
Gladwin MT, Vichinsky E
NEJM 2008; 359: 2254-65
Sickle Cell Disease
Driscoll MC
Pediatrics in Review 2007; 28(7): 259-68
Sickle Cell Disease
Graham R Sergeant
Oxford Medical Publications