Bi-Level Positive Airway Ventilation for Acute Chest Syndrome

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 (also known as BLPAP or BiPAP) is a device that blows air into a patients lungs via a mask that covers the nose. The goal of this study is to determine whether giving children BiPAP 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 BiPAP 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.

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

Eligibility Criteria:
Inclusion Criteria:

* patients diagnosed with Hemoglobin SS (HB SS), the most common type of sickle cell disease
* patients diagnosed with Hemoglobin SC (HB SC), the second most common type of sickle cell disease.
* patients diagnosed with Hemoglobin sickle beta-zero thalassemia ( HB SB0thal) or Hemoglobin sickle thalassemia (HB SBthal)

Must 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