Treatments for people with sickle cell disease in whom poor blood supply to an area of bone leads to bone death

Many people with sickle cell disease experience bone death due to temporary or permanent loss of blood supply to parts of their bones. This can be very painful. The bones usually affected are the thigh bones at the hip joint and the arm bones at the shoulder joint. The aim of treatment is to stop the pain and maintain a mobile joint. Treatments include resting the joint, physiotherapy, the use of pain relief, joint replacements and bone grafts. However, complications from surgery may be more frequent in people with sickle cell disease. We found one eligible trial which analysed data from 38 people from 32 different treatment centres. The trial compared a treatment of surgery and physical therapy to physical therapy on its own. This trial did not show that the addition of surgery to a physical therapy regimen could improve the outcome for people with sickle cell disease and avascular necrosis. However, the strength of this trial’s findings were weakened by a number of participants who could not complete the treatment. Further trials are needed which will look at the long-term outcomes of different treatments for this condition. Trialists should consider including endpoints that focus on the subjective experience of participants (such as quality of life, and pain) as well as more objective such as mortality, survival, or hip longevity. The availability of participants to allow adequate trial power will be a key consideration during endpoint choice.