Sickle Cell Disease

Pain Crisis
- CBC, pain management depending on severity may start with oral narcotics and work-up to IV/IM
- IV hydration – 1 ½ maintenance D 5 ½ NS, bolus if necessary/dehydration

Fever
- If <101 – BC, CBC, u/a + urine cx Chest Xray and pulse ox.
- If labs >28,000 WBC, Bands>5, H/H decreased from basline, CXR positive admit for IV antibiotics.

- Otherwise discharge after hematology consult.

- If temp 101-103 notify hematologist immediately, draw labs as above consider CSF cx and analysis if indicated, frequent re-evaluation, admit if any of above, other wise IM ceftriaxone and d/c home with hematology follow-up

- If temp >103 all of above work-up, IV antibiotics and admit.

Acute Chest Syndrome

Patients present with either chest pain or tachypnea/respiratory distress.
- Check O2 saturation, CXR, CBC
- Consult hematology
- Do not overhydrate, use narcotics as needed for pain to prevent splinting and atelectasis
- Coverage with antibiotic if in repiratory distress
- PICU

Transfusion Guidelines

1. Premedicate with Tylenol 15 mg/kg and Benadryl 1m/kg prior to administration of blood products.
2. PRBCs – 5 ml/kg increases Hgb by 1 gm
- Dose: 10-15 ml/kg (2 Units in adult) over 4 hr
3. Fresh frozen Plasma – useful for replacing clotting factors
- Dose 10-20 ml/kg, large volume can be a disadvantage
- Causes 15-20% rise in factor level
4. Pooled Platelets – 50-100 thousand
- Dose – 0.1-0.2 u/kg increases plt count
5. Irradiated blood products – Indications:
- Immunocompromised patients
- Oncology patients on chemotherapy
- BMT/organ transplant candidate
- Congenital immunodeficiency syndromes
- Premature infants, in utero transfusions, ill neonate
5. CMV negative products
- Filtered products are 99.9% CMV negative
- Indicated in immunocompromised individuals
- Priority (basd on availability) in
- BMT recipients if donor/recipient CMV negative
- Premature, LBW, ill neonates