Sickle Cell Disease: Pain & Fever

Sickle Cell Disease: Pain & Fever John Cheng, MD PEM Fellows’ Conference July 19, 2006

Sickle Cell Disease • Hemoglobin S • Glu  Val at 6 position of β hemoglobin • Various types: • SS • SC • Sβ-thalessemia • Others

Sickle Cell Issues • Vaso-Occlusive Crisis • Sickling and subsequent ischemia • Immunocompromise • Splenic infarction • Encapsulated organisms: H. influenzae, S. pneumonia • Salmonella

Vaso-Occlusive Crisis (VOC) • Usual type of pain? • Concerns: • Abdominal pain: splenic sequestration, gallstones • Hip pain: avascular necrosis • Headache: stroke • Chest pain: acute chest syndrome • Eye pain: optic artery ischemia • Groin pain (male): priapism • Extremity pain: dactylitis, osteomyelitis • Other pain: possible abscess

VOC--Labs • CBC with diff • Reticulocyte count • Blood cultures if h/o fever • Consider electrolytes • BMP if dehydrated • LFTs if RUQ or epigastric abd pain • Consider U/A and Ucx if abd/flank pain • Consider Type and Screen

VOC--Diagnostics • CXR if respiratory symptoms • Ultrasound--abdominal • CT scan--head

VOC--Treatment • Oxygen • Keep SaO2 ≥ 92% • May be hypoxic at baseline • Hypotonic fluids (D5 1/4NS) • Reverse sickling • Dehydration: 10 cc/kg NS bolus vs 1.5 maintenance • BEWARE fluid overload • Blood transfusion • If neeed, try to get leukocyte-depleted and, if available, C, E, Kell-compatible and sickle neg RBCs

VOC--Meds • Pain meds • NSAIDs: Ketorolac 0.5 mg/kg, max 30 mg • Opiates: • Morphine 0.1-0.2 mg/kg q 15-30 min PRN • Dilaudid 0.015-0.02 mg/kg • Mixed Opiate Agonist/Antagonist: • Nubain 0.2-0.3 mg/kg q3h PRN • Other meds: • Benadryl 1.25 mg/kg PO (NOT IV) q6 PRN

VOC--Disposition • Admission if not able to control pain OR significant drop in Hgb and/or retic • Ask if they think they can manage at home. • Home meds: • Ibuprofen 10 mg/kg q6-8h x 2d, then PRN • Tylenol #3 1 mg/kg q4-6h PRN breakthrough pain • Consider Lortab, Oxycodone, Morphine IR • Follow up with Sickle Cell clinic in 1-2 days by phone or in clinic • Call sickle cell consult.

Fever • Defined as temp ≥ 38.3°C • Immunocompromise • Splenic infarction • Usually on Penicillin until 5 y/o • Usually have PCV7 and Pneumovax • Remember to treat concurrent pain

Fever--Labs & Diagnostics • CBC with diff • Reticulocyte count • Blood cultures • Consider CRP and Type & Screen • Consider urine or CSF as warranted • Chest XRay if respiratory symptoms

Fever--Meds • No source: • GOAL: 30 minutes from door to antibiotics • Rocephin 50-75 mg/kg, max 2 gm IV/IM • If cephalosporin allergy: Meropenem 20 mg/kg IV, max 1 gm • If source found: treat as usual after IV Abx • If Acute Chest Syndrome: • Oxygen, pain meds • Consider adding Zithromax, nebulizers, and steroids

Fever--Disposition • Consider admission for observation if: • Age < 1 y/o • Previous bacteremia/sepsis • T > 40°C • WBC > 30 or < 5, plts < 100 • Received Meropenem or Vancomycin • Infiltrate on CXR • Unable to comply with follow up • Other problems: pain, aplastic crisis, splenic sequestration, ACS, stroke, priapism

Fever--Disposition • If labs unremarkable and well appearing, d/c home and f/u in 24 hours in sickle cell clinic for re-check and 2nd dose of Rocephin. • Call sickle cell consult.

CAVEAT • Read notes from previous visits. • There are some frequent flyers who are supposed to have pain plans in place with hematology.

Sickle Cell Disease: Pain & Fever