=====Sickle Cell=====
  *Sickle cell disease (SCD) and its variants - genetic disorders resulting from the presence of a mutated form of Hb, HbS.
  *most common form of SCD = homozygous HbS disease (HbSS), an autosomal recessive disorder

Pre-empting factors which may promote erythrocyte sickling (and vaso-occlusive crises) include:
  *Hypoxemia is the strongest stimulus for sickling (deoxygenated hemoglobin S is less soluble).
  *Acidosis
  *Dehydration
  *Infection and inflammation
  *Fever
  *Stasis of blood (e.g., reduced cardiac output)
==Types of sickle cell crisis presentations:==
1. fever
  *consider veno-occlusive disease, acute chest syndrome, osteomyelitis, local or systemic infection
  *vaso-occlusive crisis
  *assume this is the cause of any painful presentations
2. acute chest syndrome
  *life-threatening lung infarction
  *assume if hypoxia + chest pain
3. acute splenic sequestration
  *typically occurs in infants
  *associated with increased spleen size
  *fall in Hb by >20 g/L, thrombocytopenia but normal or increased reticulocytes
4. aplastic crisis
  *fall in Hb as well as reticulocytes <1%
  *triggered by parvovirus infection
5. stroke\\ 
6. priapism
  *stuttering (2-4h duration and may be recurrent)
  *severe (>4 hours and may lead to permanent impotence)

==Management==
Priorities:
  - Analgesia - requires aggressive pain Mx
  - oxygenation
  - rehydration - ECF (N saline)
  - may need transfusion
  - may need antibiotics
  - avoid thrombolytics in stroke
  - priapism may require surgical intervention
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===References include===
[[https://emedicine.medscape.com/article/205926-overview|Emedicine: Sickle Cell 2020]]\\ 
https://litfl.com/sickle-cell-crisis/\\ 
[[https://emcrit.org/ibcc/sickle-chest/]]