Full Title of Guideline: Guideline for Management of Acute Sickle Cell Disease. Author (include email and role): Dr Consultant Haematologist Division & Speciality: Clinical Haematology Version: 2 Ratified by: Clinical Haematology Governance Scope (Target audience, For all staff working with acute problems state if Trust wide): related directly to sickle cell patients Review date (when this October 2020 version goes out of date): Explicit definition of patient group to which it applies All adult patients with acute problems directly related to sickle cell disease. (e.g. inclusion and exclusion criteria, diagnosis): Changes from previous N/A version (not applicable if this is a new guideline, enter below if extensive): Summary of evidence base this guideline has been created from: Based on Standards of care for adults with Sickle Cell Disease in preparation. This guideline has been registered with the trust. However, clinical guidelines are guidelines only. The interpretation and application of clinical guidelines will remain the responsibility of the individual clinician. If in doubt contact a senior colleague or expert. Caution is advised when using guidelines after the review date or outside of the Trust.
Table of Contents 1 Background... 4 2 Common presentations... 4 3 Arrangements for admission at NUH (adults)... 4 4 Assessment and initial management of patient with sickle cell disease... 5 4.1 Pain... 5 4.2 Areas of concern... 6 4.3 Pain management after first 90 minutes... 7 4.4 Alternative analgesics to consider... 10 5 Investigations... 10 6 Alteration of dose of analgesia... 10 7 Oxygen... 10 8 Thromboprophylaxis... 11 9 Antibiotics... 11 10 Transfusions... 11 10.1 Exchange Transfusion... 12 11 Other acute presentation... 12 11.1 Acute chest syndrome... 12 11.1.1 Clinical features... 12 11.1.2 Diagnosis... 13 11.1.3 Differential diagnosis... 13 11.1.4 Management... 13 11.2 Abdominal pain... 14 11.2.1 Differential diagnosis... 14 11.2.2 Clinical findings... 14 11.2.3 Investigations... 14 11.2.4 Management... 15 11.3 Priapism... 15 11.3.1 Management... 15 11.4 Osteomyelitis... 15 11.5 Acute stroke... 15 11.6 Acute anaemia... 16 11.7 Acute sequestration... 16 11.8 Haematuria... 16 Page 2 of 29 Lead Author: Submission Date: October 2017 Review Date: October 2020
11.9 Acute visual loss... 17 Patients reporting acute visual loss should be advised to attend the emergency ophthalmology clinic at QMC (close to A+E and with extended evening opening hours and at weekend)... 17 12 References... 17 Appendix 1 UHL Management of Painful Sickle Cell Crisis flow chart 18 Appendix 2 Acute Painful sickle cell crisis medical management in first 60minutes... 20 Appendix 3 Acute Painful Sickle Cell Crisis nursing management in first 60 minutes... 22 Appendix 4 Example of sickle cell disease admission document... 23 Appendix 5 Sickle Cell Crisis Pathway... 26 Appendix 6 Quick Reference Guide... 27 Lead Author: Submission Date: October 2017 Review Date: October 2020 Page 3 of 29
Guideline for Adults with Sickle Cell Disease: acute presentation 1 Background Sickle cell disease affects up to 15,000 people in the UK. Many of these patients will present acutely to a variety of hospitals in the country and may not be known already to staff at the hospital. Sickle cell disease (either HbSS or compound heterozygote states Hb SC, HbS/B-thalassaemia or other compound states) commonly gives rise to acute painful crises. The pain may occur in any part of the body but is commonly in the limbs. Pain in chest or abdomen should prompt consideration of alternative causes of pain in those sites (e.g. MI, acute appendicitis) but sickle cell pain is still the most likely cause. Sickle pain is very severe usually requiring opiate analgesia, may have a pain score of up to 10/10 and it is usually clear to the patient that this is their usual sickle pain (i.e. patients with sickle cell disease are the experts in their painful crises). The pathogenesis of sickle cell pain is thought to be due to deoxygenated sickle Hb forming large polymers which cause red cells to become deformed and irreversibly sickled leading to small vessel obstruction. Triggering factors are cold, dehydration and infection but often no specific trigger is found. 2 Common presentations severe pain pyrexia, tachycardia, tachypnoea, hypotension Increased pallor, breathlessness, exhaustion chest pain, consolidation on CXR, SaO 2 < 94% on air abdominal pain or distension, diarrhoea, vomiting severe thoracic/back pain headache, drowsiness, CVA,TIA or any abnormal CNS signs 3 Arrangements for admission at NUH (adults) Patients are advised to contact the Haematology acute mobile holder (07812268298) at City hospital when possible. The patient will be told where they can be seen (haematology wards or Speciality Receiving Unit (SRU)) or in Haematology day case. Page 4 of 29 Lead Author: Submission Date: October 2017 Review Date: October 2020
If seen on SRU and requiring admission for longer than 18 hours patients will normally have to be moved to another ward: Haematology wards, Burns ward or another available bed. Patients admitted via ambulance will generally be brought to City hospital but some patients (not known to service, self-presentation or with other issues which may be more appropriately managed in QMC) may be seen in A+E at QMC. If the patient is thought to have a sickle cell crisis they will be transferred to City hospital by ambulance after appropriate analgesia. In all cases the Haematology SpR must be informed (however an uncomplicated admission in the middle of the night could be discussed first thing the next morning) 4 Assessment and initial management of patient with sickle cell disease The general guide for these patients is to speedily assess them if they are known to the service (have ID cards from NHR, or have previous annual review or admission document in the document section of NotIS OR electronic patient record) then assess pain and observations speedily. 4.1 Pain If the pain is usual sickle cell pain and is severe (often patients will have tried their usual oral analgesia before), observations are stable and there are no unusual features analgesia should be given as before: check previous drug charts or dose as follows: Morphine 0.1 mg/kg IV repeated every 20 minutes until pain controlled. Analgesia must be administered within 30 minutes of admission (NICE CG143) If this IV dose is administered by a doctor, suggest diluting 10mg in 10ml saline (1mg/ml) giving 5mg and then titrating to effect over the next 5-10 minutes. Patients with no opioid tolerance may need up to 20mg. Patients with tolerance may need a higher starting dose and a higher total dose. Laxatives, antiemetics and thromboprophylaxis (unless contraindication) should be prescribed. Note baseline monitoring and do bloods (FBC, retics, U+Es, LFTs, CRP, LDH, Clotting, Group and save, Blood cultures if fever or suspicion of infection initially. Full admission clerking should be completed within 2 hours of admission) Page 5 of 29 Lead Author: Submission Date: October 2017 Review Date: October 2020
THEN: inform Haematology SpR, ask nursing staff to monitor every 30mins (BP, pulse, pain, temp, O 2 sats, respiratory rate, sedation) until pain controlled and then 2 hourly and put on fluid balance chart. Patient will usually require IV fluids, oxygen, thromboprophylaxis, analgesia regime, laxative and perhaps antiemetic and antipruritic. 4.2 Areas of concern Fever, neurological symptoms, priapism, swollen limbs, complaint of chest or abdominal pain, possibility of pregnancy Please see simplified SRU flow chart (appendix 2 & 3) for simplified guidance for first 60 minutes of admission, Leicester flow chart (appendix 1) for further guidance and appendix 4 for an example of admission document which it is hoped to provide for each sickle cell patient (they may carry a copy and a copy should be available on NotIS/electronic patient record) Entonox may be available in SRU. Those transferring from paediatric care may be treated with oral morphine and intranasal diamorphine is becoming available on paediatric areas and this may be available on the adult side. Those transferring from paediatric clinic and previously receiving oral analgesia should be continued on this Involvement of the Acute Pain Team if ongoing concern about pain relief is recommended Lead Author: Submission Date: October 2017 Review Date: October 2020 Page 6 of 29
4.3 Pain management after first 90 minutes Time from 1 st assessment 0-90mins Reason Action Comments Immediate control acute pain Morphine 0.1 mg/kg IV repeated every 20 minutes until pain controlled Can give SC >90mins >90mins Continued intermittent analgesia-administered by staff using an appropriate pain chart and checking every 30 minutes till pain controlled and at least every 4 hours thereafter PCA (patient controlled analgesia) If this IV dose is administered by a doctor, suggest diluting 10mg in 10ml saline (1mg/ml) giving 5mg and then titrating to effect over the next 5-10 minutes. Patients with no opioid tolerance may need up to 20mg. Patients with tolerance may need a higher starting dose and a higher total dose. THEN continued analgesia administered by staff or PCA. Morphine 0.1 mg/kg IV or SC every 2-4 hours. Extra doses if pain persistent (50% of given dose) after 1 hour. OR Morphine 1 mg/ml solution administered IV (PCAs at City currently fixed at 1mg/ml concentration) Background: 2-3 mg/hour (i.e. 2 to 3ml/hr) PCA bolus dose: 1-3mg/bolus Page 7 of 29
Dose duration:1 minute Every 30mins then every 1 hour for 6 hours and at least every 4 hours thereafter Monitor Lockout time:20-30 minutes Pain, sedation, vital signs, respiratory rate until pain controlled and stable then every 4 hours Reduced respiratory rate <10 per minute omit opioid analgesia (usually <8) Extra pain relief Prevent vomiting If severe respiratory depression <6 give 100 micrograms naloxone IV repeated every 2 min as necessary Give high flow oxygen by face mask 1) Paracetamol can be given IV in acute phase dose 1g over 15mins up to four times daily, maximum dose 4g 2) Ibuprofen, Diclofenac if no contraindication to NSAIDs 3) Local heat 4) TENS machine CONSIDER ALTERNATIVE DIAGNOSIS Prochlorperazine 5-10mg tds or cyclizine 50mg tds (avoid prolonged parenteral cyclizine use) Suggest use of paracetamol and NSAID unless contraindicated Optional Page 8 of 29
Ondansetron 4mg oral or s/c Laxative Lactulose 10ml bd, Senna 2-4 tablets bd, docusate Essential 100mg bd Antipruritic Hydroxyzine 25mg bd Optional Page 9 of 29
4.4 Alternative analgesics to consider Oxycodone, Ketamine, Methadone, fentanyl are alternatives but their use should always be discussed with a consultant and the Acute Pain team unless specifically indicated in that patient s documentation. 5 Investigations Patients presenting with sickle cell crisis require the following investigations: FBC and reticulocyte count U&E and LFT Blood cultures MSU Oxygen saturation (ABG if < 95% on air) Lp (if CNS symptoms, discuss with experienced haematologist first) Group and save Check %HbS if on transfusion regime or %HbF if on hydroxycarbamide The following may be required: CXR if chest signs/symptoms, (SO2 <95%, dyspnoea, cough, chest pain) XR is indicated if a patient develops localised bone pain and fever as osteomyelitis is a recognised and potentially serious complication of sickle cell disease CRP 6 Alteration of dose of analgesia Requirements for analgesia needs to be reconsidered on a daily basis at least when pain is well controlled aim to reduce dose by small amounts and switch to oral equivalents when patient is clearly improving (pain score / chart may be helpful) 7 Oxygen There is no good evidence for this being used routinely in all cases of painful crisis Page 10 of 29
Its use should be dictated by the clinical situation and oxygen saturations: If SaO 2 <95% on air, give O 2 by face mask Check arterial gases if SaO 2 on air is <95% Monitor SaO 2 while patient is on supplementary oxygen aiming to keep O 2 level > 98% If arterial po 2 (PaO 2 ) <10.7kPa use mask giving 35% inspired oxygen If arterial PaO 2 < 9.3kPa seek additional help generally by involving HDU outreach team 8 Thromboprophylaxis Sickle cell disorders are associated with an increased thrombotic potential All patients who are admitted with a severe sickling crisis who are immobile should be commenced on low molecular weight heparin prophylaxis Do not use TED stockings 9 Antibiotics If the patient is febrile or has a history suggestive of an infective cause of the sickle crisis they should be commenced on antibiotics Augmentin is the antibiotic of choice in most cases unless penicillin allergic Most patients are on oral penicillin maintenance which can be stopped and restarted when the course of Augmentin has been completed 10 Transfusions See Transfusion guideline for further guidance Patients with sickle cell disease often have a chronic anaemia of 80-100 g/l which is normal for them Hb S has a lower oxygen affinity than Hb A so tissue oxygenation is better than expected It is useful to check the patient s steady state Hb level (eg from an OPD appointment) when reviewing their blood count The Hb may fall 10-20 g/l during a sickle crisis but blood transfusion is NOT routinely indicated and in fact may exacerbate a crisis Page 11 of 29
Blood transfusions should only be used if there are symptoms of severe anaemia, or if the Hb has fallen >20g/l or is below 50 g/l. The transfusion should aim to return the Hb to the steady state level and the blood should be matched for Rhesus and Kell antigens, be sickle negative and as fresh as possible 10.1 Exchange Transfusion The aim of an exchange transfusion is to replace the sickle blood with Hb AA Exchange transfusion should be discussed with a Consultant The indications for urgent exchange transfusion are: o Sickle chest crisis o Cerebral sickling/acute ischaemic stroke o Multi-organ failure Red cell exchange is preferably done using the cell separator via the stem cell nurses who should be contacted on Ext 57054 or via the Haematology Day Case Unit Rarely emergency exchanges may have to be performed manually if necessary out of hours Routine elective exchange transfusion may be done for recurrent severe crises or pre-operatively Manual exchange transfusion may be necessary in an emergency (there will be a pack near specialist nurse desk on Fletcher ward) Blood should be Rhesus and Kell genotyped for the patient and Blood Bank should be contacted as soon as an exchange transfusion is planned Hb A and S levels should be sent to the haematology laboratory after exchange transfusion A Hb S level of <30% is the goal of this therapy 11 Other acute presentation 11.1 Acute chest syndrome This is a life threatening complication of sickle cell disease and can arise during a painful crisis or occur on admission 11.1.1 Clinical features Some of the following may be present: chest pain (may be pleuritic) cough Page 12 of 29
shortness of breath hypoxia: oxygen sats <94% or more than 3% below baseline fever tachypnoea wheeze tachycardia fall in haemoglobin Chest signs (some or none may be present): crepitations bronchial breath sounds reduced air entry dullness to percussion rhonchi 11.1.2 Diagnosis clinical suspicion CXR: new pulmonary infiltrate, sometimes normal arterial blood gasses FBC, U+Es, reticulocyte count, clotting screen, %Hb S (if recent transfusion), CRP, group and antibody screen Microbiology: blood and sputum culture, atypical serology, sputum viral DNA PCR culture, urine for legionella and pneumococcal antigens, nasopharyngeal aspirate if coryzal 11.1.3 Differential diagnosis acute infection PE opiate toxicity fluid overload under-ventilation due to pain 11.1.4 Management vigilance (4 hourly monitoring of pulse oximetry and respiratory rate increased to at least hourly if clinical suspicion of ACS) Early liaison with HDU / outreach team as transfer may be necessary involve Haematology SpR / Haematology Consultant Page 13 of 29
TRANSFUSION: simple top up especially if Hb decreased from baseline (post transfusion Hb <10); manual or automated exchange is preferable if deteriorating patient, widespread infiltrates, if top-up likely to result in increased viscosity (or limited response to top up), other problems (see transfusion guidelines) aim O2 saturation >96% chest physio check on appropriate broad spectrum antibiotics may need microbiology advice hydration essential but avoid fluid overload (use fluid chart) pain control must be continued with regular monitoring Nebulised bronchodilators may be helpful continue thromboprophylaxis, change dose if clinical suspicion of PE incentive spirometry may be helpful 11.2 Abdominal pain 11.2.1 Differential diagnosis cholelithiasis pancreatitis referred pain from chest appendicitis acute splenic/hepatic sequestration Infection constipation ischaemia of bowel thrombosis e.g. renal vein vaso-occlusion 11.2.2 Clinical findings tenderness lack of/heightened bowel sounds 11.2.3 Investigations FBC, retics, U+Es, LFTs, coag, lipase, blood cultures, MSSU plain abdominal X-ray abdominal ultrasound / CT as guided by radiology Page 14 of 29
11.2.4 Management surgical opinion may prove useful but acute surgical intervention is rarely indicated discuss with senior haematologist maintain good hydration (generally IV fluids) close monitoring IV antibiotics if febrile (and with a view to covering likely GI pathogens) early recourse to ITU referral / outreach team 11.3 Priapism Two types: stuttering (recurrent episodes lasting <30 mins) or fulminant (>3 hours). Both are associated with pain and fulminant priapism is a medical emergency. Fulminant priapism may also occur in context of painful crisis in other body part. This is difficult to discuss with patients you may have to ask (or look in annual review records) 11.3.1 Management hydration, analgesia, encourage micturition etilefrine 50mg may be given by mouth urgent urology (on call at CITY hospital) opinion should be sought if priapism persists after 3-4 hours then aspiration of corpora cavernosa (with or without irrigation) or intracavernous injection of dilute phenylephrine transfusion therapy is not thought useful in acute presentation 11.4 Osteomyelitis consider in any patient with hot painful limb as part of differential diagnosis of painful crisis x-ray not always helpful but should be done if osteomyelitis is considered include blood cultures for any febrile patient choice of broad spectrum antibiotics should include cover for likely pathogens (seek microbiology advice) 11.5 Acute stroke may be ischemic or haemorrhagic in adults diagnosis needs to be confirmed radiologically urgently Page 15 of 29
thrombolysis should be considered in sickle cell patients with acute cerebral thrombosis this should be discussed by the senior haematologist and the stroke physicians transfusion therapy should be given in order to reduce HbS to <30% but timing will require discussion those with subarachnoid haemorrhage may have multiple aneurysms 11.6 Acute anaemia presentation with pallor, extreme lethargy possible underlying pathologies: parvovirus infection, sequestration (see below), folate deficiency investigations: FBC and retics, parvovirus serology, urgent cross match (where possible genotyped and sickle negative) usual steady state Hb values may be helpful (from clinic visits) 11.7 Acute sequestration sudden enlargement of spleen (occasionally liver) in which blood is pooled in the organ leading to a severe reduction in circulating red cells and profound anaemia abdominal palpation will reveal an enlarged spleen urgent FBC and retics followed by urgent cross match (if possible genotyped and sickle negative red cells) as a top up transfusion 11.8 Haematuria Acute haematuria can be due to renal papillary necrosis and can be accompanied by ureteric colic (if obstructive) and can be associated with significant blood loss. The diagnosis may be confirmed on ultrasound but sometimes a CT scan is required. Treatment is supportive and occasionally urological support may be required for renal irrigation. Other causes of haematuria need to be borne in mind (e.g. renal cancers) Page 16 of 29
11.9 Acute visual loss Patients reporting acute visual loss should be advised to attend the emergency ophthalmology clinic at QMC (close to A+E and with extended evening opening hours and at weekend) 12 References 1. Rees et al (2003) Guidelines for the management of the acute painful crisis in sickle cell disease. British Journal of Haematology; 120: 744-752. 2. National Institute for Health and Care Excellence (2012) Sickle cell acute painful episode. CG143. London: National Institute for Health and Care Excellence. 3. Lottenberg R, Hassell KL. (2005) An evidence based approach to the treatment of adults with sickle cell disease. Hematology; 58-65 4. Standards for the clinical care of Adults with sickle cell disease (2017) in preparation Page 17 of 29
Appendix 1 UHL Management of Painful Sickle Cell Crisis flow chart See following page Page 18 of 29
Developed by C. Chapman, Z. Suleman & M. Aheame Weight Kg Doctor to complete Is Pain adequately controlled after 20 mins? 3. Complete Initial Assessments 4. Assess Urine output and commence fluid balance 5. Take sample of urine +/- send for MC&S 6. Antibiotics to be given without delay in cases with acute sepsis (refer to UHL antimicrobial website) 7. Hydration please provide jug of water and encourage oral fluids to prevent further sickling. Inform doctor if patient unable to tolerate as patient will need IV fluids 8. Ensure patient is warm, provide extra blankets if necessary 9. Weigh the patient 10. Repeat the observations every 30 MINS until pain is resolved. Investigations Blood FBC, U&E, reticulocytes, LFT, CRP G&S +/- cultures Urine +/- MC&S Imaging CXR if dyspnoea, Sp02 <95%, fever, cough, chest pain Sickle Cell Disease: Painful crisis Patient Details Name: Guideline flowchart for Adults with Sickle Cell Disease: Acute Presentation DOB: To be followed when a patient with sickle cell disease presents Hosp No: to A&E unwell ED Nurse or Doctor to complete 1. Assessment of Pain & EWS score 2. Administer analgesia using WHO analgesic ladder and see box A Y Check Respiratory Rate every hour 1. Take history including Pain/infection/neurology/surgical history/vaccination/ precipitating event/recent transfusions 2. Examine inc CVS/RESP/Abdo/Neuro/ENT/Skin assess for signs of septic shock, pneumonia, CHF, acute chest syndrome / likely sources of infection 3. Investigations 4. VTE assessment +/- Dalteparin 5. Obtain IV access (avoid cannulating legs) Is the patient high risk? (see box C) N Any Red Flag Events? (see box D) N Assess Opiate Toxicity (see box B) Y Y A. Management of Pain Mild Pain: Paracetamol 0.5g -1g QDS +/- Codeine 1mg/kg +/- Ibuprofen 200mg TDS OR Diclofenac 50mg BD Moderate/Severe Pain: Add Morphine 100mcg/kg SC or IV repeat after 20 MINS if necessary. Then 50-100mcg/kg every 2-4 hours OR continuous infusion via PCA B. Are there signs of opiate toxicity? Likely if the following present Low Respiratory Rate : RR < 12 Hypoxia : Oxygen saturation SP02 <94% OR <9kPa PO2 Drowsiness Pin Point pupils If none of these present Safe to give another dose of morphine C. Is the patient high-risk? Previous ITU admission Previous blood / exchange transfusion? D. Red Flag Events Acute Chest Syndrome Aplastic Crisis (suspect if Hb < 2 units below baseline and reticulocytes are low/normal Stroke Priapism Acute Abdomen Splenic Sequestration Contact Haematology SpR via switchboard Contact Bed Bleep on 45XX if admission required Name of SpR:... Advice Given: Blood Transfusion Advised? Yes/No If Yes: Order Sickle negative blood Not for Blood transfusion unless discussed with Haematology SpR 6. Manage Pain according to Box A. 7. Patients unable to tolerate oral fluids need IV fluids (10-20mls/kg of 0.9% normal saline. Followed by maintenance fluids. Caution in patients with renal/cardiac problems. Close monitoring is advised. 8. Check blood results Page 19 of 29 N Is Pain adequately controlled? Y 9. Check if safe for discharge see box E N E. Safe for Discharge? Haematology Registrar Contacted? Yes/No Is Pain Controlled Yes/No Last morphine dose administered 4 hours ago Y/N EWS is 0 Yes/No If EWS > 0 have you discussed with Haem SpR Y/N If ALL answers are yes patient safe for discharge
Appendix 2 Acute Painful sickle cell crisis medical management in first 60minutes Triage as high priority Confirm diagnosis where possible (check photo- ID card if patient has one, check NotIS, notes) Initial Assessment: Site of pain Whether usual sickle pain and severity (use pain chart if possible) Ask about analgesics used, usual hospital analgesia and any allergies Check baseline monitoring (BP, Pulse, temp, O2 sats, respiratory rate) Give analgesia: usually opiates but if difficulty in establishing IV access subcutaneous or oral route might be preferred (buccal fentanyl could also be used) Must be given within 30 minutes of arrival. May need repeated if unsatisfactory relief When patient more comfortable check history further and examine (if either incomplete at this stage please complete within 2 hours of admission), note baseline monitoring and do bloods (FBC, retics, U+Es, LFTs, LDH, Clotting, Group and save, Blood cultures if fever or suspicion of infection) THEN: inform Haematology SpR, ask nursing staff to monitor every 30mins (BP, pulse, pain, temp, O2 sats, respiratory rate, sedation) till Page 20 of 29
pain controlled and then 2 hourly and put on fluid balance chart. Patient will usually require IV fluids, oxygen, thromboprophylaxis, analgesia regime, laxative and perhaps antiemetic and antipruritic Areas of concern: fever, neurological symptoms, priapism, swollen limbs, complaint of chest or abdominal pain, possibility of pregnancy 2017 Page 21 of 29
Appendix 3 Acute Painful Sickle Cell Crisis nursing management in first 60 minutes This is an acute medical emergency and such patients need attention and support as soon as they arrive in the department do baseline observations (BP, pulse, O2 sats, respiratory rate, pain score) doctor needs to be informed immediately of sickle cell patient with acute crisis in department reassure patient, ask site of pain and type (? like usual sickle pain) if there is opportunity prepare for IV access, fluid balance chart, pain chart (more copies available from resource file or sickle cell specialist nurse) needs to have observations checked as above and sedation level every 30 minutes until stable and pain free and then 2 hourly fluid balance must be checked and noted on chart Alert doctor to: chest and abdominal pain, priapism, high fever, neurological symptoms, swollen limbs or pregnancy Doreen Richards 2015 Page 22 of 29
Appendix 4 Example of sickle cell disease admission document See following page. Page 23 of 29
TO BE USED FOR NAMED PATIENT ONLY for PAINFUL SICKLE CRISIS Patient Name: #### ########## Hospital no: ####### NHS no: ### ### ##### Dob: 28/2/1984 Diagnosis: Hb SS Date of completion: 20/11/12 Date of review: 2014 Please see Sickle cell flow chart and guidance if unsure ask Haem SpR or contacts below Issue Usual analgesia for sickle crisis in Hospital* Other treatment Particular issues /complications Allergies Usual analgesia at home Other medication need for monitoring (Eg Hydroxycarbamide) Usual follow -up Management /comment Morphine 5-10 mg initially SC or IV, further 5mg every 20 minutes until pain controlled 5-10mg be repeated every 2 hours Fluids encourage at least oral intake of 1 litre every 8 hours otherwise IV may be required Enoxaparin if admitted (unless contraindication) Antiemetic Laxative Supplemental oxygen if sats <96% Pain Chart Previous weight loss please monitor Check! Tramadol None Please encourage op attendance * DO NOT GIVE OPIATES IF RESPIRATORY RATE <9, OXYGEN SATS <90% or drowsy seek medical advice first. Please let Haematology SpR know of admission. Further guidance may also be obtained from Dr Donohue (bleep 6822, sarah.donohue@nuh.nhs.uk) Doreen Richards (bleep7972, doreen.richards@nuh.nhs.uk) or sickle cell service (0115 883 8424) Audit Time of arrival Time of first analgesia Pain score initially Pain score at 90- Page 24 of 29
TO BE USED FOR NAMED PATIENT ONLY for PAINFUL SICKLE CRISIS Patient Name: #### ########## Hospital no: ####### NHS no: ### ### ##### Dob: 28/2/1984 Diagnosis: Hb SS Date of completion: 20/11/12 Date of review: 2014 Please see Sickle cell flow chart and guidance if unsure ask Haem SpR or contacts below Monitoring as per guideline Other medication Other complications Bp, O2sats, p, temp, resp rate Laxative, LMWH etc 120min Any learning points? Oct 12 Page 25 of 29
Appendix 5 Sickle Cell Crisis Pathway Sickle Cell Crisis - Acute Chest Syndrome : Management Pathway and Referral Pathway to the HDU and ITU Presentation with respiratory signs and symptoms (as below in the table) including hypoxia +/- new CXR infiltration Fever ++ Tachypnoea + Cough ++ Wheezing +/- Chest pain ++ Skeletal pain ++ Dyspnoea ++ Haemoptysis+ A high clinical suspicion is vital to early diagnosis Acute Chest Syndrome can be a severe life-threatening condition Stable patients can deteriorate rapidly to acute respiratory failure Think ahead of Exchange Transfusion and ITU input Investigations CXR, FBC, UEs, LFTs Blood group and screen (or crossmatch) Blood cultures Sputum cultures ABG Serology/urine for atypical respiratory organisms SpO2 95% (on air) on pulse oximetry or a fall in SpO2 of 3% or more from baseline steady state confirmed with ABG promptly Management Fluids, Pain relief, Oxygen, Antibiotics +/- bronchodilators Contact and inform on-call Consultant Haematologist for advice Above investigations may need to be repeated in the deteriorating patient Inform Blood Bank early on about possible blood transfusion requirement Exchange Transfusion need to be done on HDU/ITU and protocol is available from Haematologist Falling po2 Hb<70g/dl or po2(air) <9.0kPA Hb>70g/dl And/or Rapid deterioration plus indicators of severity po2(air) <8.0kPa pco2 >6.0kPa Top up transfusion ITU outreach team review Exchange transfusion ITU review Consideration of NIV/IV Page 26 of 29
Appendix 6 Quick Reference Guide EMSCT guidance on sickle cell disease acute management Good Practice Reference- to national /local guidelines Acute pain o Rapid assessment (basic NUH Guidance page 5 observations, check with patient) o Pain relief within 30 minutes - e.g. morphine 0.1mg/kg parenterally or patient has specific pain plan o Continued monitoring every 30 mins till pain relieved then 2 hourly o Then fuller assessment and investigations (FBC retics, u+es, LFTs, Gp and save, LDH, blood cultures if fever, haemoglobinopathy screen if indicated), CRP o ADD laxative, thromboprophylaxis, paracetamol, NSAID unless contraindicated o Iv fluids if insufficient oral intake o Supplemental oxygen if <SO2 <95% Fever o Blood cultures and MSSU, other cultures if symptoms Identify cause and treat within local guidance o Low threshold for antibiotics o Suspend penicillin V if on broad spectrum antibiotics Monitor CRP NUH Guidance page 6 Chest syndrome o Warning signs : chest pain, falling NUH Guidance page 12 oxygen saturation, SOB o Do CXR (look for widespread infiltrate but may be normal) o Check blood gasses o Consider HDU/ ITU transfer o Exchange transfusion Page 27 of 29
Priapism o If prolonged can be medical emergency (exercise, warm bath can help) o Analgesia o Early discussion with urology if >2hours, may need to aspirate o Alpha adrenergic blocker e.g. etilefrine may help o Transfusion therapy probably not helpful Neurological o Acute ischaemic or haemorrhagic stroke possible o Urgent imaging Acute sequestration Acute anaemia Page 28 of 29 o Thrombolysis indicated as for nonsickle patient o Urgent red cell exchange for ischaemic stroke? also for haemorrhagic o Sudden enlargement of spleen (occasionally liver) with blood pooling resulting in profound anaemia o Urgent blood transfusion required o Symptomatic anaemia (SOB, lethargy, pallor) with Haemoglobin usually falling by 25% of baseline or less 20g/l from steady state level o Top up transfusion may be necessary o Exclude other causes (e.g. parvovirus, bleeding, folate deficiency) Osteomyelitis o Consider in any febrile patient with swollen painful limb o Xray may not be helpful especially initially o Use broad spectrum antibiotics as per Trust guidance Haematuria o If painless may be papillary necrosis (pain only if accompanied by obstruction) o Monitor loss NUH Guidance page 15 NUH Guidance page 15 NUH Guidance page 16 NUH Guidance page 16 NUH Guidance page 15 NUH Guidance page 16
Visual loss o Us may be helpful, otherwise CT o If persistent, bladder irrigation may be helpful o NB other causes of haematuria o Any acute symptoms should be seen urgently - eye casualty at QMC NUH Guidance page 17 Page 29 of 29