DIAGNOSIS, GUIDELINES FOR COMPREHENSIVE CARE, AND CARE PATHS AND PROTOCOLS FOR MANAGEMENT OF ACUTE AND CHRONIC COMPLICATIONS  the Sickle Cell Disease Care Consortium (Arizona, Colorado, Georgia, Missouri, New Mexico, Tennessee, Texas, and Utah)

ACUTE SPLENIC SEQUESTRATION

IN CHILD WITH SICKLE CELL DISEASE

DEFINITION: An acute illness associated with hemoglobin (Hb) 2 gm/dl or more below patient's baseline value with acutely enlarged spleen. Mild to moderate thrombocytopenia is often present. Reticulocytosis equal to or greater than baseline is usually present. If reticulocyte count is decreased, consider coexistent aplastic crisis 

CONSULT

Hematology

MONITORING:

1. Hospitalize

2. Consider ICU admission for signs of cardiovascular compromise.

3. Vital signs q 2 hr until stable, then q 4 hr.

4. CR monitor

5. Continuous pulse ox

6. Record I+O, daily weight

7. Serial exams (initially q 2-4 h) to reassess cardiovascular status and spleen size

DIAGNOSTICS:

1. CBC, diff, platelet count, and reticulocyte count initially, then q 4-12 hr depending on severity of anemia, rate of fall in Hb level, changes in spleen size.

2. Type and crossmatch RBC stat. Time permitting, consider if available minor-antigen-matched, sickle-negative, and leukocyte-depleted RBC.

3. Blood culture, urinalysis, and urine culture if febrile. Consider CSF and other cultures.

4. Consider CXR if febrile or if any signs or symptoms of respiratory illness present.

FLUIDS, GENERAL CARE:

1. IV + PO @ 1 X maintenance. More fluids may be needed if insensible losses are increased (e.g. persistent fever) or to support

intravascular volume before transfusion.

2. Incentive spirometry - 10 breaths q 2 hr when awake if on parenteral narcotics.

MEDICATION/TREATMENT:

1. RBC transfusions 10 cc/kg for Hb <4-5 gm/dl and/or signs of cardiovascular compromise. Transfusion may be needed for Hb <7-8 gm/dl for patients with relatively high baseline Hb levels (e.g. HbSC disease). In severe cases, urgent initiation of transfusion prior to inpatient admission may be life-saving. A post-transfusion hemoglobin level of £ 8-9 gm/dl is generally recommended to avoid the risk of hypervicosity that may occur several days later when red blood cells sequestered in the spleen may return to the circulation and increase the hemoglobin 1-2 gm/dl above post-transfusion levels.

2. Cefotaxime or cefuroxime 50 mg/kg IV q 8 hr if febrile. Substitute clindamycin 10 mg/kg IV q 6 h for patients with known or suspected cephalosporin allergy. Strongly consider adding vancomycin 10-15 mg/kg IV q 8 hr for severe febrile illness or for proven or suspected CNS infection.

3. If applicable, continue prophylactic penicillin. Penicillin prophylaxis should be discontinued while patient is receiving broad-spectrum antibiotics.

4. 0₂ by nasal cannula or face mask if needed to keep pulse ox ³ 92% or ³ patient's baseline value, if >92%. The etiology of a new or increasing supplemental 0₂ requirement should be investigated. 0₂ @ 2 liters by nasal cannula or 35% by face mask can be given empirically for the severely anemic child who is to receive RBC transfusions.

5. Acetaminophen 15 mg/kg po q 4 hr (maximum daily dose 75 mg/kg) and/or ibuprofen 10 mg/kg po q 8 hr for any fever and/or mild pain. (Hyperthermia may exacerbate cardiovascular compromise with severe anemia.)

6. Morphine sulfate 0.05-0.15 mg/kg IV q 2 hr or 0.05-0.1 mg/kg/hr continuous infusion or via PCA for severe pain. (For PCA give 1/3-1/2 of total maximum dose by continuous infusion and 1/2-2/3 via PCA boluses.) Total morphine dose, continuous infusion plus boluses, above 0.1 mg/kg/hr may occasionally be required but should be used with caution. Alternative analgesics including hydromorphone (Dilaudid) 0.015-0.02 mg/kg IV q 3-4 hr may be appropriate in selected cases.

7. See other Clinical Care Paths for vaso-occlusive pain, acute chest syndrome, aplastic crisis, stroke, priapism, if present.

DISCHARGE CRITERIA:

1. Stable hemoglobin/hematocrit.

2. Taking oral fluids well and able to take po medications (e.g. prophylactic penicillin) if applicable.

3. Afebrile ³ 24 hr. and negative cultures for ³ 24-48 hr. if applicable.

4. Adequate pain relief, if needed, with oral analgesics.

5. Follow-up arranged.

6. Consider surgical splenectomy and/or chronic transfusions for severe or recurrent events.

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References

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Last modified: November 01, 2002