Emergency Presentations of Sickle cell disease. Dr S Pancham Sickle Cell & Thalassaemia Centre City Hospital

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1 Emergency Presentations of Sickle cell disease Dr S Pancham Sickle Cell & Thalassaemia Centre City Hospital

2 Aims Key features to elicit at presentation What complications to look for When to phone for advice

3 Distribution of patients Greater London 8555 West Midlands 1368

4 West Midlands adult haemoglobinopathy network GEOGRAPHICAL REGIONAL SERVICE MAP Stoke SHROPSHIRE STAFFORDSHIRE Shrewsbury and Telford Stafford Wolverhampton WORCESTERSHIRE Russells Hall Worcester Walsall BIRMINGHAM Heartlands QE Coventry

5 Sickle Cell Presentations Vasocclusive crisis Complications Sepsis -gallstone complications Acute sickle chest -priapism Acute stroke Sequestration syndromes Any medical problem Distinguish from usual VOC

6 History to probe, assessing the phenotype What is the usual pattern of his/her SCD? Is this person in hospital often? What with? What complications has he/she had? ITU admissions Transfusion history End organ damage Any sites for chronic pain Where is he/she receiving specialist care? Does the history flag any concerns?

7 Full assessment to include Full obs NB oxygen saturation on air Any fever significant? Use local eg sepsis tool, MEWS General eg Hydration Chest review Abdomen liver, spleen Any neurological signs? detailed if suspect stroke Review specifics eg leg ulcers, vascuport or Hickman line site

8 FBC and retics SS S/β o thalassaemia S/β + thalassaemia SC Investigations g/l g/l g/l g/l U&E, LFTs CRP Group and screen Sepsis screen Viral serology if appropriate Should a CXR be done? Should an ABG be done? XR limbs?

9 Analgesia Most patients would have tried simple analgesia at home; opiates needed in this setting Analgesia as per local guideline Aim quick & effective analgesia- time to first dose of analgesia 30 mins (NICE standard) Review efficacy with the patient

10 Analgesia Morphine usually first option Options for morphine allergy- oxycodone, fentanyl Pethidine not recommended risks inc seizures, muscle abscesses, contractures PCAs Entonox prolonged use not recommended Monitor RR, saturations, pain score Very few patients with SCD have opiate dependency

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12 General measures Fluids O2 VTE prophylaxis eg enoxaparin Folic acid 5mg

13 Case 1 18 yr F Hb SS Mild to moderate phenotype Admitted with pain lower limbs, ribs Sats 95% on air Chest clear

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15 Day 3 of admission Generally unwell Short of breath on exertion Coughing up yellow sputum

16 Acute Chest Syndrome Clinical or radiological evidence of consolidation Usually with chest pain, fever, dyspnoea, wheezing, cough Tachypnoea, tachycardia, hypoxaemia Second commonest cause for admission after VOC Severity: self limiting to rapid progression to acute respiratory failure

17 Acute Chest Syndrome Presentation: Adults >80% skeletal pain at presentation Children may be presenting diagnosis Aetiology: Fat/marrow embolism 20% Infection 50% mostly atypical bacteria and viruses Infarction Sequestration

18 Management of acute chest syndrome Analgesia O2 Fluids Urgent cross match Early ITU review Nebulised salbutamol Antibiotics (include macrolide) Transfusion support

19 Acute anaemia Increase in haemolysis Bone marrow suppression Sequestration syndromes Red cell aplasia Delayed transfusion reactions

20 Transient red cell aplasia Preceding viral symptoms Most commonly due to human parvovirus B19 infection Cessation of red cell production 4-8 days Transfuse for severe anaemia Monitor non immune contacts -any siblings with SCD, pregnant staff Transfuse to baseline Hb

21 Sequestration syndromes Acutely unwell abdominal signs Hepatic sequestration Acute tender hepatomegaly Obstruction of sinusoidal outflow Conjugated hyperbilirubinaemia Usually associated with infection esp salmonella

22 Splenic sequestration 45 yr old Hb SS Tender splenomegaly Intermittent episodes of anaemia past year

23 Sepsis Low grade pyrexia can accompany uncomplicated VOC Leucocytosis common with VOC Immune dysfunction Hyposplenism Penicillin prophylaxis? Sites to consider chest, urine, biliary tract, bone, infected leg ulcer

24 Case 2 22 yr old Hb SS Fever 39 0 C and Rt shin swelling x 4 days CRP 138 Sterile blood cultures

25 Case 2 Intramedullary BM oedema over a length of approx. 20cm in the middle third of the right tibia. Cortical thickening of bone Consistent with osteomyelitis. IV Ceftriaxone x 6 weeks

26 Case 3 19 yr old Hb SS Back pain atypical for sickle and fever Recent travel to Tunisia Salmonella in BC

27 Priapism Form of vaso-occlusive crisis affecting penile circulation Prolonged sustained unwanted painful erection with/ without prior sexual stimulation

28 Types of priapism Stuttering < 1 hour Less severe More frequent Resolves Estimates 50% patients Median age of onset 12 years Acute/ fulminant >1 hour Up to several days 2/3 preceding stuttering priapism Untreated can lead to infarction of erectile tissue Fibrosis, impotence long term

29 Management of acute priapism Emergency- contact urologists Do not pack priapism in ice Effective analgesia Hydration Urinary retention?- catheterise if necessary Moderate exercise to create steal syndrome Contact haematologist for further management

30 u mo l /L Case 4 20 yr Hb SS Acute abdominal pain & fever Differentials? Bilirubin (umol/l) Bilirubin May H Jun Jul Aug Sep Oct Nov Dec Jan 2014 Feb Mar Apr Saviour, Kenneth

31 Hepatobiliary complications Gallstones Sequestration Intrahepatic cholestasis Iron overload Infections

32 Aetiology of stroke Generalized brain atrophy in rare cases borderzone infarction Small white matter lesions visible on MRI Large cerebral infarcts Subcortical infarction Intraventricular and intraparenchymal haematoma Rare venous system clots Arteriolar thickening and capillary dilatation Cortical vessel dilatation Lenticulostriate arteries form moyamoya Proximal arterial stenosis at ICA, MCA, ACA Most common site of occlusive disease is here Ophthalmic artery Subarachnoid haemorrhage from aneurysm rupture Aneurysms, often multiple on circle of Willis Fat embolism Dilatation of vertebrobasilar system due to collateral flow Cervical ICA usually unaffected

33 Transfusions in the acute setting To improve anaemia and oxygen carrying capacity To reduce Hb S % Top up transfusions- acute anaemia or symptomatic anaemia Exchange vs top up transfusion therapy When to exchange? How to exchange?

34 Blood Transfusion Specific indications Each patient has unique degree of anaemia Acute anaemia- top up transfusion Emergency indications- acute stroke, acute chest syndrome Complications of transfusion Allo antibodies in 30% of patients Hyperhaemolysis

35 Questions