Dr Abdul Mannan: The Hidden Emergency in Sickle Cell Disease
Dr Abdul Mannan, Consultant Haematologist at Betsi Cadwaladr University Health Board, posted on LinkedIn:
“When Minutes Matter: The Hidden Emergency in Sickle Cell Disease
Yesterday, a 2-year-old with sickle cell disease presented with sudden abdominal pain and pallor. Within hours, his hemoglobin dropped from 8.5 to 4.2 g/dL. The culprit? Acute splenic sequestration crisis – the most life-threatening emergency in sickle cell disease.
Unlike typical pain crises, sequestration demands immediate recognition. Blood pools rapidly in the enlarged spleen, causing cardiovascular collapse. The key is cautious transfusion: 3-5 mL/kg aliquots, checking hemoglobin after each dose. Why? Sequestered cells return to circulation as the crisis resolves, risking dangerous hyperviscosity if we overtransfuse.
Key takeaway: Early family education on spleen palpation saves lives. Recurrence rates reach 78%, making prevention education critical.”
Read more here.
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