Sickle cell patients often find difficulty obtaining adequate treatment of pain. Anti-inflammatory medications, steroids, and opioids can reduce pain; however their use is limited by side effects. We present a 12 year old female with sickle cell disease who was admitted with severe right thigh pain secondary to acute infarction of the proximal femoral diaphysis.She was treated with intravenous hydration, ketorolac and opioids with no relief. The patient agreed to a continuous femoral nerve block, which was done under sedation. Pain relief was obtained immediately. The infusion was discontinued after 3 days and the patient was discharged the day after catheter removal with a one week supply of fentanyl patches and home dose of oral oxycodone. We suggest continuous femoral nerve block as an adjunct to treat sickle cell pain crises involving the lower extremity. Regional techniques may vasodilate and thus improve the nociceptive, vasoconstrictive pain associated with acute pain crises.
Although most patients with sickle cell disease first attempt to treat their acute pain episodes at home, acute pain crises are the most common cause for seeking medical attention in this population [
We present a 12-year-old, 38 kg African American female with hemoglobin SS disease who was admitted with her typical sickle cell crisis pain. She had attempted to manage 24 hours of 10/10 pain at home with both oral ibuprofen and oxycodone without relief. She continued to describe severe, throbbing, non-radiating right thigh pain which persisted despite treatment with 0.6 mg iv hydromorphone and 17.5 mg iv ketoralac. After admission to the pediatric hematology service, laboratory evaluations revealed no infectious etiology and electrolytes and blood counts were within normal limits, including hemoglobin 12.1. She was given iv hydration, and for pain control she was continued on 17.5 mg iv ketoralac every 6 hours with hydromorphone pca 0.2 mg basal rate with bolus 0.2 mg every 15 minutes as needed, in the setting of a history of intolerance to morphine which caused shortness of breath and a pruritic rash. Within a few hours of the dilaudid pca initiation, the patient developed a diffuse pruritic rash relieved with diphenhydramine, but the pca was discontinued and she was switched to oxycontin 10 mg q 12 hours and oxycodone 5 mg q 4 hours as needed, with iv dilaudid as breakthrough relief. MRI of her right leg revealed acute infarction of the proximal femoral diaphysis and reactive myositis along the medial and lateral aspects of the femoral diaphysis. She received multiple doses of intermittent hydromorphone without adequate relief, whereupon the acute pain service was consulted.
After discussion with patient, family, and primary service, a femoral nerve block was agreed upon, utilizing a catheter to provide continuous ropivicaine infusion. However, secondary to anxiety, the patient and family requested sedation for placement of the catheter. This was done uneventfully under propofol infusion 80 mcg/kg/ minute with standard ASA monitors and a nasal cannula with end tidal CO2 monitoring, titrated to oxygen saturation of ≥98% in a post-anesthesia acute care bed. We used ultrasound guidance as well as a nerve stimulator to place the femoral nerve catheter under sterile technique and infused a single dose of 8 cc of 2% lidocaine for immediate relief. The catheter was sterilely dressed and an infusion pump was programmed for the continuous ropivicaine infusion. The patient recovered from the procedural sedation of 30 minutes without issues, vital signs stable, and was returned to her floor bed with a 0.2% ropivicaine infusion run over the following 3 days. The infusion rate was adjusted from 7 ml/h to 3 ml/h secondary to concerns of numbness, and by day 3 the infusion was discontinued after sufficient pain control using fentanyl patch at 50 mcg/h and oral oxycodone, home dose. On day 4, after her pain had been controlled, the catheter was pulled, and the patient discharged to home with one week of fentanyl patches and oxycodone.
Acute pain control in sickle cell crisis should involve consideration of regional anesthetic techniques. An acute pain crisis is often described as primarily nociceptive rather than neuropathic pain, as they are often vaso-occlusive episodes involving ongoing ischemia and infarction [