Clinical features

A common presenting symptom is the so-called 'hand and foot syndrome' which occurs in early infancy and is characterised by painful dactylitis. Later in life the bone pain is widespread and particularly marked in the back and limbs. The pain is frequently bilateral and symmetrical; it tends to be close to joints and can be mistaken for pauci-articular arthritis.

Acute painful episodes are characterised into 4 distinct phases:

• prodromal phase (pre-crisis):
  • patients feel numbness, aches and parasthesia in the sites which will be affected by pain
  • can last for two days
• initial infarctive phase:
  • characterised by the onset of typical crisis pain
  • the pain increases gradually with a peak around the second or third day
  • infarction from sickling of red blood cells was thought to be responsible for the pain episodes although newer evidence suggest a much more complicated pathophysiology
• post-infarctive phase:
  • there is persistent severe pain
  • signs and symptoms of inflammation are seen mostly
• resolving (post-crisis) phase:
  • pain slowly resolves over one to two days (1)

Reference

1. NICE. Sickle cell disease: managing acute painful episodes in hospital. Clinical guideline CG143. Published June 2012