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

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

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

4. 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

5. 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

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

7. 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

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

9. 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.

10. 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

11. 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

12. Fever--Meds No source: If source found: treat as usual after IV Abx
GOAL: 30 minutes from door to antibiotics
Rocephin 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

13. 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

14. 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.

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