Haem: Sickle-Cell Anaemia: Free MSRA Podcast

🎧MSRA Podcast: SickleCell Anaemia – What Every Clinician Needs to Know! 🎧Get ready for anengaging, clear, and clinically-focused deep dive into sickle cell anaemia—from genetics to day-to-day management,classic exam features, and essential red flags. Fast, memorable, andexam-ready! 🗝️ Key Learning PointsDefinitionSickle cell anaemia = inherited blood disorder: abnormal HbS (sickle haemoglobin) causes red cells to become stiff, sickle-shaped, and prone to blocking blood vessels.Result: vaso-occlusion (painful crises), chronic anaemia, organ damage.Causes & Risk FactorsAutosomal recessive inheritance (both parents carry the HbS gene)Most common in those of African, Caribbean, Middle Eastern, and South Asian heritageTrait (HbAS) = carrier, usually no symptomsDisease (HbSS) = two abnormal genes, classic symptomsPathophysiology Mnemonic: SICKLES: S**ubstitution in HBB geneI: Impaired red cell flexibilityC: Chronic haemolysisK: Kidney & other organ damageL: Low oxygen triggers sicklingE: Episodes of pain (crises)Clinical FeaturesPainful crises (bones, chest, abdomen)Chronic anaemia (fatigue, pallor, jaundice)Infection risk (non-functioning spleen)Delayed growth (children), leg ulcers, gallstones, priapismComplications: stroke, acute chest syndrome, avascular necrosis, kidney/lung/eye problemsDiagnosisNewborn screening (heel prick test)FBC: anaemia, ↑ reticulocytesBlood film: sickle cells, target cells, Howell-Jolly bodiesGold standard: haemoglobin electrophoresis / HPLCRapid test: Sickle solubility screen (screening only)Genetic testing as neededRed Flags / When to Seek Urgent CareSevere pain not settlingFever >38.5°C or signs of infection/sepsisChest pain, breathlessness (acute chest syndrome)Neurological symptoms (stroke signs)Priapism >2 hours, sudden pallor, splenic enlargementManagementPrevention:Penicillin V (up to age 5+), vaccinations (pneumococcal, flu, Hib, MenC, etc.)Avoid triggers: dehydration, cold, stress, hypoxiaAcute crises:Pain relief (stepwise: paracetamol/NSAIDs → opioids)Hydration, oxygen, treat infection if presentBlood transfusion for severe anaemia/complicationsDisease modification:Hydroxyurea (↑ HbF)Exchange transfusion (for stroke/acute chest)Crizanlizumab for recurrent crises (over 16s, NICE criteria)Curative:Bone marrow/stem cell transplant (rare, children, matched donor)Psychological and social support essential!Complications – Mnemonic: PAINSP: Painful crises / PriapismA: Acute chest syndrome / Avascular necrosisI: Infections (sepsis, osteomyelitis)N: Neurological (stroke, learning difficulties)S: Splenic dysfunction, Stunted growthPrognosisImproved with early diagnosis, prophylaxis, comprehensive careMedian life expectancy in UK: 40–60+ yearsEarly intervention, hydroxyurea, and regular specialist review are key to best outcomes 📝 MSRA Revision ResourcesRevision Notes:https://www.passthemsra.com/topic/sickle-cell-anaemia-revision-notes/Flashcards:https://www.passthemsra.com/topic/sickle-cell-anaemia-flashcards/Accordion Q&A:https://www.passthemsra.com/topic/sickle-cell-anaemia-accordion-qa-notes/Rapid Quiz:https://www.passthemsra.com/topic/sickle-cell-anaemia-rapid-quiz/Online Quiz:https://www.passthemsra.com/quizzes/sickle-cell-anaemia/ #MSRA#SickleCellAnaemia #Haematology #MSRARevision #PassTheMSRA #PainfulCrises#AcuteChestSyndrome #UKGuidelines #MedStudent #MedEd Exam Pearl:Pain + anaemia + infection risk in a patient ofAfro-Caribbean or African heritage?Always consider sickle cell anaemia and act fast onred flags!