> [!references]-
> [RCH - Sickle Cell Disease](https://www.rch.org.au/clinicalguide/guideline_index/Sickle_cell_disease/) - [devonthink](x-devonthink-item://A0A462AC-D316-4E7A-8D67-F3105FBBF62E)
> [dunn - sickle cell](x-devonthink-item://6D1B09B8-D427-4D59-A75A-7871797F67C8)
> [Rosen - sickle cell disease](x-devonthink-item://EC6083F0-5243-4221-8B3C-C78DD78C2EA2?page=13&istart=2093&ilength=18&search=Sickle%20Cell%20Disease)
> [RMH Sickle Cell Disease - Acute and Perioperative](x-devonthink-item://14B791EB-CFCB-4F70-9A0A-5300E5B5B741)
> [!key points]
> - Avoid [[IV contrast]] → generally discuss with haem if required
> - Likely transfuse RBC if Hb <100
> - low threshold for broad spectrum abx
# Investigations
- FBC, reticulocyte count, blood film
- +/- Hb electrophoresis
- Fe studies
- +/- virology (eg parvovirus B19)
Ensure vaccinated in accordance with functional asplenia guideline
# sickle cell crisis
- vaso-occlusive crisis
- usually a stressor -- infection, dehydration, or cold -- causes sickle cells to lodge in the microcirculation
- **bone marrow infarction** is a well-recognised complication of it, but can happen anywhere
- symptoms:
- spinal pain
- abdo pain (mesenteric occlusion of girdle sequestration)
- chest pain (pulm vascular occlusion)
- joint pain
- fever (secondary to tissue necrosis)
- neuro (TIA, strokes, seizures)
- [[Penile aspiration|priaprism]]
- "hand and foot syndrome" (dactylitis of infancy)
- haematuria (nephrotic syndrome, papillary necrosis)
- skin ulcers
- retinopathies
- gallstones
# Treatment
- analgesia eg morphine
- supplemental O2
- IV fluids if renal involvement
- abx cover
- exchange transfusion if neurological presentation, lung involvement, sequestration syndromes, priaprism
## Hydroxyurea
- consider fertility conselling prior to starting
- Iam for HbF >10%
## Acute erythrocytapheresis
> - Usually every 4-6 weeks using 4-6 RBC units.
> - Aims to reduce HbS to <30% acutely and maintains HbS <50% prior to next exchange transfusion
### Indications
- visceral ischaemia
- acute chest syndrome
- stroke
- intracranial haemorrhage
- liver failure
- retinal occlusion
- priaprism
- sepsis
- multiorgan failure
- acute pain syndrome not responding to standard analgesia
# acute chest syndrome
Sickle cell disease can produce an acute illness related to infarction of the lung tissue. Usually associated with lower respiratory symptoms, hypoxaemia and a new infiltrate on CXR. Chest pain and hypoxaemia may be the only signs.
Chest pain should be treated as an acute chest syndrome and not simply as a vaso-occlusive crisis.
**NB** This is a life threatening illness and patients may deteriorate quickly
> - look for CXR changes, hypoxia.
> - consider PE or empirical anticoagulation
# Other acute syndromes
- aplastic crisis
- acute abdomen → consider **acute splenic sequestration** if shock in young patient and usual surgical causes
- stroke → d/w haem re: contrast
- Iron overload