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Early Noninvasive Ventilation and Nonroutine Transfusion for Acute Chest Syndrome in Sickle Cell Disease in Children: A Descriptive Study

Heilbronner, Claire, MD1; Merckx, Audrey, MD1; Brousse, Valentine, MD2; Allali, Slimane, MD2; Hubert, Philippe, MD1; de Montalembert, Mariane, MD, PhD2; Lesage, Fabrice, MD1

Pediatric Critical Care Medicine: May 2018 - Volume 19 - Issue 5 - p e235–e241
doi: 10.1097/PCC.0000000000001468
Online Clinical Investigations

Objectives: To describe the need for transfusion and short- and long-term evolutions of pediatric sickle cell disease patients with acute chest syndrome for whom early continuous noninvasive ventilation represented first-line treatment.

Design: Single-center retrospective chart study in PICU.

Setting: A tertiary and quaternary referral PICU.

Patients: All sickle cell disease patients 5–20 years old admitted with confirmed acute chest syndrome and not transfused in the previous month were included.

Interventions: None.

Measurements and Main Results: Demographic data, laboratory and radiologic findings, transfusions, invasive ventilation, oxygen and noninvasive ventilation settings, duration of opioid treatment, length of hospital stay, and severe sickle cell disease complications in the ensuing 2 years were extracted from medical charts. Sixty-six acute chest syndrome in 48 patients were included. Continuous early noninvasive ventilation was well tolerated in 65 episodes, with positive expiratory pressure 4 cm H2O and pressure support 10 cm H2O (median) administered continuously, then discontinued during 7 days (median). No patient necessitated invasive ventilation or died. Twenty-three acute chest syndrome (35%) received transfusions; none received blood exchange. Transfused patients had more frequent upper lobe radiologic involvement, more severe anemia, higher reticulocyte counts, and higher C-reactive protein than nontransfused patients. Their evolution was more severe in terms of length of opioid requirement, length of noninvasive ventilation treatment, overall time on noninvasive ventilation, and length of stay. At 2-year follow-up after the acute chest syndrome episode, no difference was observed between the two groups.

Conclusions: Early noninvasive ventilation combined with nonroutine transfusion is well tolerated in acute chest syndrome in children and may spare transfusion in some patients. Early recognition of patients still requiring transfusion is essential and warrants further studies.

1Pediatric Intensive Care Unit, AP-HP, Hôpital Necker Enfants Malades, Paris, France.

2Department of Pediatric, AP-HP, Hôpital Necker Enfants Malades, Paris, France.

Dr. Brousse’s institution received funding from Addmedica, and she received funding from Novartis. Dr. de Montalembert’s institution received funding from Novartis, and she received funding from Addmedica. The remaining authors have disclosed that they do not have any potential conflicts of interest.

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©2018The Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies