Bi-Level Positive Airway Ventilation for Acute Chest Syndrome

• Rate of exchange transfusions. [ Time Frame: Diagnosis until discharge. Average 7 days. ] [ Designated as safety issue: No ]
• Determine parent and patient acceptability of BLPAP administration in the setting of ACS. [ Time Frame: Upon completion of intervention at 48hrs. ] [ Designated as safety issue: No ]
• Rate of PCCU transfers. [ Time Frame: Diagnosis until discharge. Average 7 days. ] [ Designated as safety issue: No ]
• Difference in respiratory rate. [ Time Frame: 48hrs after intitiation of treatment. ] [ Designated as safety issue: No ]
• Difference in pulmonary function tests. [ Time Frame: 48hrs after intitiation of treatment. ] [ Designated as safety issue: No ]
• Difference in mean SpO2 recording during sleep. [ Time Frame: From initiation of treatment to 48hrs. ] [ Designated as safety issue: No ]

Acute chest syndrome (ACS) is a frequent complication of sickle cell disease and is diagnosed by having findings on a chest x-ray and one of the following: chest pain, fever, or trouble breathing. Patients with Acute Chest Syndrome can get very sick and require an exchange transfusion (special large blood transfusion) and mechanical ventilation. Bi-level Positive Airway Pressure (BLPAP) is a device that blows air into a patients lungs via a mask that covers the nose. Our goal is to determine whether giving children BLPAP when they have ACS, in addition to providing standard clinical care for ACS alters the clinical course of these patients. The investigators hypothesize that patients receiving effective BLPAP will have milder clinical courses resulting in shorter hospital stays and fewer transfers to the intensive care unit and exchange transfusions.

Acute chest syndrome (ACS) is a frequent complication of sickle cell disease and is diagnosed by a new infiltrate on chest x-ray and one of the following: chest pain, fever, or respiratory signs or symptoms (tachypnea, cough, new onset hypoxemia, or increased work of breathing.)The treatment for acute chest syndrome is focused on supportive care with hydration, antibiotics, blood transfusions and respiratory support. Unfortunately, despite these treatments many patients fail to have improvements in their respiratory status, or have respiratory decompensation. These patients require more aggressive treatments, which frequently include exchange transfusions, pediatric intensive care unit (PCCU) management, and respiratory support.

Our goal is to perform a prospective double blind randomized control trial to investigate if early initiation of effective BLPAP in addition to providing standard clinical care for ACS alters the clinical course of these patients vs. sham BLPAP and standard clinical care. We hypothesize that patients receiving effective BLPAP will have milder clinical courses resulting in shorter hospital stays and fewer transfers to PCCU and exchange transfusions.

• Sickle Cell Anemia
• Acute Chest Syndrome

• Procedure: Bi-level positive airway pressure
  BLPAP initiated for at least 16 hours per day for a minimum of 48hrs.
• Procedure: Sham CPAP
  Sham CPAP initiated for at least 16 hours per day for a minimum of 48hrs.

• Experimental: Bi-level Positive Airway Pressure
  BLPAP initiated for at least 16 hours per day for a minimum of 48hrs.
  Intervention: Procedure: Bi-level positive airway pressure
• Sham Comparator: Sham CPAP
  Physiologic CPAP initiated for at least 16 hours per day for a minimum of 48hrs.
  Intervention: Procedure: Sham CPAP

Inclusion Criteria:

Hemoglobin SS, SC, SB0thal or SBthal Ages ≥ 4-21 years old Patients are admitted to the hematology oncology inpatient service and have been enrolled and consented in the study.

Meet clinical criteria for ACS- an infiltrate on Chest X-ray and one of the following:

• Respiratory symptoms/signs (patients pulse oximetry < 92% or oxygen saturation < 2% below their baseline, tachypnea, cough, and increased work of breathing)
• Fever
• Chest pain AND

Patients' eligible for a simple transfusion based on one of the following criteria:

• Hypoxemia (patients pulse oximetry < 92% or oxygen saturation < 2% below their baseline)
• Hemoglobin < 5 gm/dl
• Increased work of breathing

Exclusion Criteria:

• Patient requires exchange transfusion within first 24 hours of admission
• Patient requires PCCU transfer within first 24 hours of admission
• Hemoglobin > 9gm/dl secondary to these patients requiring an exchange transfusion