Sickled Simon — Sickle Cell Vaso-Occlusive Crisis
Severe bone pain, dactylitis, priapism risk — hydrate, oxygenate, treat pain around the clock
Simon, age 7, wakes screaming with pain in his legs and back after playing outside in cold weather without a jacket. He has had sickle cell disease since infancy and knows this pain well.
Simon has sickle cell disease, and cold, dehydration, infection or hypoxia trigger his red blood cells to sickle and clog small vessels. A vaso-occlusive crisis causes severe bone and joint pain, and in infants swollen painful hands and feet (dactylitis). Aggressive IV fluids, oxygen if hypoxic, and scheduled (not PRN-only) opioid analgesia are the priorities, alongside watching for acute chest syndrome, the leading cause of death in sickle cell disease.
Read Sickled Simon's full story →The story arc
The setup
Simon plays outside on a cold, windy day and comes home dehydrated and shivering, then complains his legs ache.
The turn
By evening the ache has become throbbing pain in his long bones, and he rates it 8 out of 10, refusing to walk.
The crisis
In the ED his pain is unrelenting despite one dose of ibuprofen, his respiratory rate climbs, and he develops chest pain with a mild cough.
The save
The nurse starts IV fluids, administers scheduled IV opioids around the clock rather than waiting for pain to peak, applies supplemental oxygen, and notifies the provider immediately about his chest symptoms.
NCLEX study section — Sickle Cell Vaso-Occlusive Crisis
What this chapter is testing: the recognition cues, the numbers and the priority action.
Study section: Pediatrics
Take this chapter into practice questions on the same topic.