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.