Guidelines for the treatment of sickle cell disease-related pain in hospitalized children

Pain is the most common morbidity experienced by children with sickle cell disease (SCD). When outpatient pain management strategies fail, two-thirds of patients seen in the emergency department (ED) will ultimately require inpatient admission. Many of the common strategies used to treat hospitalized children with vaso-occlusive pain episodes are based on available clinical evidence and experience; limited practical guidelines exist for the inpatient management of pain in SCD. We identified supporting evidence for the use of selected opioid analgesics, scheduled intravenous (IV) pain medications, patient controlled analgesia (PCA), weaning from maximum dose with conversion to equianalgesic oral pain medications, and use of incentive spirometry during inpatient management of acute painful episodes. Despite the perception of the benefit, no evidence could be identified and no expert opinion supported the use of rapid infusion of intravenous (IV) fluids (boluses), blood transfusions, prophylactic oxygen, antihistamine therapy, and ketorolac or methadone administration for the management of acute SCD pain. In several instances, significant toxicities have been identified with these therapies that have marginal to no proven benefit for the treatment of inpatient painful episodes in children with SCD. Establishing a standard pain management strategy that is consistent, transparent and easy to follow is an important for optimal inpatient management of pain among children with SCD. Clinical strategies that accentuate treatment with a firm pharmacological basis and eliminating therapy that has no proven efficacy will ultimately improve the quality of care for this vulnerable patient population.