Download Inpatient Sickle Cell Disease Acute Chest Syndrome

Children’s Hospital of Pittsburgh
Guidelines for Clinical Effectiveness
Inpatient Sickle Cell Disease Acute Chest Syndrome Guideline
Fever, cough, dyspnea, wheezing, chest pain
•Begin Pain Management Immediately
•Patients should be treated according to their individualized management plan (Refer to
ED binder).
History




Obtain and document associated symptoms
Review any oxygen requirement, obtain baseline Oxygen sats.
Review history of asthma, current medications, drug allergies
Review medical history focusing on sickle cell disease, history of acute chest syndrome
and pneumonia
Laboratory Evaluation
CBC (including WBC diff and platelet)
Rapid HgbS level
Chemistry profile
Liver function tests
Type and Screen (sickle cell negative-leukocyte depleted)
PRBC’s, Rh (C,E,) and Kell antigen-matched blood. (Patients with prior history of
allosensitization should receive PRBCS that are phenotypic ally identical.)
• Consider ABG based on clinical condition (Severe hypoxemia PaO2 < 70 mm Hg indicates
potentially life-threatening disease.)
•
•
•
•
•
•
Transfusion Therapy
• Hct < 30 %= Red cell transfusion
• Multiple lobe involvement, PaO2 < 70 mm Hg= Perform an exchange transfusion
• Early transfusions are indicated for patients at high risk for complications.
Monitoring





Continuous pulse oximetry
Vital signs with blood pressure at least every 4 hours
Consider cardio-respiratory monitor if suspect over-sedation
Chest x-ray daily
Accurate Intake and Output
Guideline developed by Lakshmanan Krishnamurti, MD & staff
Acute chest syndrome (ACS) is defined by the appearance of a new pulmonary
infiltrate on chest X- ray with fever, chest pain or respiratory symptoms
(tachypnea, wheezing, cough)
Management
•
•
•
•
•
•
•
•
•
Oxygen to maintain sats ≥ 94%
Bronchodilators
Chest X-ray daily and with clinical deterioration
Incentive spirometry every 2 hours while awake, every 4 hours while
sleeping.
Consider chest physiotherapy
HCT <30, give simple transfusion –improves survival & multi-lobe
involvement. Goal: Hbg 10, HCT 30.
ABg<70, perform exchange transfusion – respiratory failure likely
Hydration using oral and IV fluids. Total hydration should not exceed 11 ½ times maintenance.
I/O and weigh daily
Medications
• Analgesics for pain control (refer to pain guideline)
• Appropriate antibiotic combination of cephalosporin (Ceftriaxone) and
macrolide (Azithromycin)- see formulary for dosing details.
• Consider adding Vancomycin if clinical deterioration
• Continue prophylactic penicillin
• Tylenol 10-15 mg/kg/dose for fever
• Laxatives for narcotic-induced constipation.
• For puritis administer antihistamines such as diphehydramine or
hydroxyzine
• Antiemetics for nausea
Discharge home with previous pain control regime when meets
the discharge criteria of:
•
•
•
•
Sats ≥ 94% on room air or baseline O2 requirement
Afebrile for at least 24 hours
Adequate oral intake, including medications
Adequate pain relief (if needed) with oral analgesics
CE # 210.05
Version # 1
Origination: 4/2006
Revised:
Next Revision: 2008
This clinical guideline is a collaborative care plan and is not intended to construed or to serve as a standard of medical care. Rather, it is intended as a guideline to promote coordination and communication with
respect to patient care and may be modified to meet individual care needs. For additional information contact the Department of Care Coordination at 412/692-7743
©Children’s Hospital of
Pittsburgh, 2006