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

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

Sickle Cell Issues Vaso-Occlusive Crisis Immunocompromise 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 Hypotonic fluids (D5 1/4NS) Blood transfusion 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 Other 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: If source found: treat as usual after IV Abx 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.