A child with sickle cell and - PDF document

1 A child with sickle cell and PIMS - TS
A child with sickle cell and PIMS - TS Jonathan Broad Paeds ST3 Debbie Sobande Paeds Registrar 10 year old boy in the Emergency Dept Presentation 2/7 Pain back, chest, thigh Afebrile on arrival No cough/D+V/dysurea/ muco - cutaneous ch

2 ange Off food/drink No unwell contacts B
ange Off food/drink No unwell contacts Background Sickle cell anaemia (ss) Takes hydroxyurea, PenV, folic acid, abidec Previous painful crises Enuresis ongoing Epistaxis 2019 Osteomyelitis 2014 Initial assessment 8/10 pain, obs normal Exa

3 mination normal Hb 100g/L (baseline) Plt
mination normal Hb 100g/L (baseline) Plt 145 x10^9, Lymph 2.1, Neutr 6.6, CRP 6mg/L, WCC, U+Es, LFTs normal CXR normal Sickle crisis + Difficult pain management Pain management Encourage oral hydration Consider imaging if change Monitor for f

4 ever Progression of illness D1 - 2 Pain
ever Progression of illness D1 - 2 Pain management Requiring PCA & clonidine D 3 Fever ?Source HR rising; prior CRP rising 63 Abdomen pain Commenced Ceftriaxone + Increased macrogol D4 PIMS - TS? Fever 38.5 Abdomen pain persists Thigh/ches

5 t/back pain Examination: Abdomen tender
t/back pain Examination: Abdomen tender LIF Tender hip but full ROM Investigations Virology Covid PCR neg ; serology positive [later] Bacteriology Blood + urine culture negative Cardiology ECG normal, ECHO emerging coronary changes Bloods

6 : Hb 88 g/L WCC 11.4 Lymph 0.4,Neutr 7
: Hb 88 g/L WCC 11.4 Lymph 0.4,Neutr 7.3x10 9, Ferritin 880 ug /L Ddimer 8.5 mg/L Na 133 mmol /L Albumin 39 g/L CRP 266 mg/L Xray abdomen: constipation Case definition (RCPCH) 1. Persistent fever, inflammation, organ dysfunction 2. Exclusi

7 on of other microbial cause 3. +/ - SARS
on of other microbial cause 3. +/ - SARS - CoV - 2* Additional features Lethargy and Myalgia Abdominal pain/Diarrhoea/Vomiting Rash/Conjunctivitis Hypotension Raised CRP Raised Ferritin �(500) Lymphopenia / Neutrophilia Raised Fibrinoge

8 n Raised D - Dimer Platelets initially l
n Raised D - Dimer Platelets initially low or normal Raised Troponin and B - NP Renal dysfunction *Not present at time Case definition (RCPCH) 1. Persistent fever, inflammation, organ dysfunction 2. Exclusion of other microbial cause 3. +/ -

9 SARS - CoV - 2 * Additional features Let
SARS - CoV - 2 * Additional features Lethargy and Myalgia Abdominal pain/Diarrhoea/Vomiting Rash/Conjunctivitis Hypotension Raised CRP Raised Ferritin �(500) Lymphopenia / Neutrophilia Raised Fibrinogen Raised D - Dimer Platelets initi

10 ally low or normal Raised Troponin and
ally low or normal Raised Troponin and B - NP Renal dysfunction *Not present at time 2.Sickle crisis +Painful thighs 3 .Sepsis? 4 . Abdominal pain 1. IVIG 2g/kg Methylpred 10mg/kg Tocilizumab D2 of diagnosis Aspirin LD Dalteparin Colecalcife

11 rol 100,000 IU 2. Analgesia Hydroxyur
rol 100,000 IU 2. Analgesia Hydroxyurea 3. Continue ceftriaxone 4 . Omeprazole Laxatives increase Movicol BD Surgical review [nil concern] 1.PIMS TS Investigations and progress 0 100 200 300 400 D1 D2 D3 D4 D5 D6 D7 D8 CRP (mg/L) 0 50 100 1

12 50 Hb (g/L) 0 20 40 Trop (ng/L) 0 20 40
50 Hb (g/L) 0 20 40 Trop (ng/L) 0 20 40 D1 D2 D3 D4 D5 D6 D7 D8 Ddimer (mg/L) 35 36 37 38 39 D1 D2 D3 D4 D5 D6 D7 D8 Fever (highest/d) Tocilizumab Methylpred IVIG Progression of illness D5 - D6 Apyrexic 24hrs MRI showed chronic avascular necr

13 osis + bone infarcts not septic Epistax
osis + bone infarcts not septic Epistaxis �20mins Hb 70 D7 Chest pain + palpitation T wave inv ECG leads 1 - 3 Trop & BNP rise CTPA - no PE Coronary dilation D8 morning Abdo + leg pain persistent Blood transfusion PIMS TS + Coron

14 ary artery dilatation + Chest pain/ palp
ary artery dilatation + Chest pain/ palpitations + Sickle crisis + Painful thigh + Epistaxis with Hb drop + Sepsis? + Abdominal pain / constipated D8 Clinical Deterioration ● Drowsy �� lower GCS ● New onset headache + vomiti

15 ng ● Hypertension ● New Left facial
ng ● Hypertension ● New Left facial droop ● New Left sided weakness in upper and lower limbs Impression and Initial Management • ? Raised ICP secondary to stroke • Neuroprotective measures including intubation • Transferred arra

16 nged to PICU ● Start Levetiracetam â—
nged to PICU ● Start Levetiracetam ● Stop Dalteparin , aspirin and sedation CT Brain Rt sided Haemorrhagic stroke frontal lobe with midline shift Current situation Update from Kings’ 13/06 - Craniotomy, surgical evacuation, ICP monitori

17 ng - Transfused peri - operatively - Sl
ng - Transfused peri - operatively - Slow improvement in GCS off sedation - ACA + MCA infarction on CT head - Extensive saphenous vein+ IVC thrombus �� IVC filter - Spiking temperature with raised CRP: IV Pip - taz , off steroids

18 CT Brain Infarcts ACA/ MCA region Conclu
CT Brain Infarcts ACA/ MCA region Conclusion Difficult case - 10 yo with sickle crisis - Addition to + complicated by - PIMS - TS - H aemorrhagic stroke - Extensive thrombi Challenges Difficult questions - PIMS vs sepsis vs sickle crisis - Abd

19 ominal pain differentials - Difficulties
ominal pain differentials - Difficulties in anticoagulation - Sickle management in Covid and PIMS - TS illness Thanks! + Any questions? Acknowledgements: ● Paediatrics ● Haematology ● PID ● Rheumatology ● Cardiology ● PICU ● Nur