Therapy

• Give oxygen if hypoxic ^a  

• Consider broad-spectrum antibiotics ^d
• Ensure an adequate fluid intake ^d
• Seek expert advice ^d

Painful crises 

• Give analgesia
• Give morphine or diamorphine iv or im regularly ^a or via patient-controlled analgesia ^d  
  • Avoid regular administration of pethidine
  • Give regular NSAIDs ^a  
  • Try TENS if the pain is bad  ^d  

Acute chest syndrome

• Give analgesia
• Use incentive spirometry ^a 
  
  Ask patients to breathe as deeply as possible 10 times every 2 hours from 8 am to 10 pm, and at night if awake until their chest pain settles 

Priapism

• Give analgesia
• Aspirate the corpus cavernosum and irrigate with dilute adrenaline. ^c

  • Following a local anaesthetic injection, aspirate the corpus cavernosum using a 23 G needle, syringe and 3 way tap. ^c

  • Then irrigate using 10 ml of 1:1,000,000 solution of adrenaline (i.e. 1 ml of 1:1000 adrenaline in 1 litre 0.9% saline).

  • Apply firm pressure for 5 minutes.
• Proceed to early surgery if there is no resolution. ^d

Consider exchange blood transfusions if any of the following are present:  

• lung involvement ^c  
• sequestration syndromes ^d
• neurological involvement (stroke, TIA, fits)  ^d

There is no clear benefit from:

• steroids ^d
• cognitive coping therapy  ^d
• anti-sickling agents, e.g. cetiedil ^d
• purified poloxamer ^d