PAIN CRISES IN PATIENTS WITH SICKLE-CELL DISEASE

About 70% of all patients with sickle cell disease suffer from pain cr ises. Pain crises are recurrent episodes of pain that range in severit y from mild to severe, usually occur very abruptly and are often local ized around joints. Pain crises are caused by vaso-occlusions in the v ascular bed of the bone marrow, leading to necrosis, edema and increas ed pressure. For effective analgesia morphine or morphine analogues ar e often required. When treating a pain crisis the patient's complaints need to be taken seriously and analgesic therapy should be started pr omptly with analgesics in proportion to the severity of the patient's pain. With mild pain oral non-opioid analgesics are sufficient, in mod erate pain they are given in combination with oral codein. Severe pain requires IV morphine, also combined with a non-opioid analgesic. Intr avenous morphine makes a thorough monitoring of ventilation and level of consciousness mandatory. Sickle cell patients do not become drug de pendant if given morphine for adequate analgesia. While bone marrow tr ansplantation has become an accepted treatment modality for sickle cel l patients with severe pain crises, treatment with hydroxyurea to incr ease HbF levels and reduce incidence and severity of pain crises, howe ver, is still experimental.