Bi-Level Positive Airway Ventilation for Acute Chest Syndrome

Not Applicable

Terminated

Interventions: Device: Sham CPAP
Device: Bi-level positive airway pressure device

Brief Summary: 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.

Detailed Description: 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.

Recruitment & Eligibility

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

Arm && Interventions

Group	Intervention	Description
Sham CPAP	Sham CPAP	Physiologic continuous positive airway pressure (CPAP) initiated for at least 16 hours per day for a minimum of 48hrs.
Bi-level Positive Airway Pressure Device	Bi-level positive airway pressure device	BiPAP initiated for at least 16 hours per day for a minimum of 48hrs.

Primary Outcome Measures

Name	Time	Method
Length of Stay as Measured by the Time From Initial Diagnosis of ACS Until Meeting Discharge Criteria.	From diagnosis of ACS until meeting discharge criteria- Average 7 days.	Length of stay as measured by the time from initial diagnosis of ACS until meeting discharge criteria. It is anticipated length of stay will correlate to efficacy of treatment: shorter stay is theorized to indicate more efficient treatment.

Secondary Outcome Measures

Name	Time	Method
Determine Parent and Patient Acceptability of BLPAP Administration in the Setting of ACS.	Upon completion of intervention at 48hrs.
Difference in Pulmonary Function Tests.	48hrs after initiation of treatment
Difference in Respiratory Rate.	48hrs after initiation of treatment
Rate of Exchange Transfusions.	Diagnosis until discharge. Average 7 days.
Difference in Mean SpO2 Recording During Sleep.	48hrs after initiation of treatment	Peripheral capillary oxygen saturation (SpO2) is an estimate of the amount of oxygen in the blood. It is the percentage of haemoglobin containing oxygen compared to the total amount of haemoglobin in the blood (i.e. oxygenated haemoglobin vs oxygenated and non-oxygenated haemoglobin).
Rate of PCCU Transfers.	Diagnosis until discharge. Average 7 days.