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SNAP: Supportive noninvasive ventilation for acute chest syndrome prevention in children with sickle cell disease
Cara S Guenther1, Victoria J Pae1, Caitlin M Neri1,2
1Department of Pediatrics, Boston Medical Center, Boston, Massachusetts, USA.
Insights
Noninvasive bi-level positive airway pressure ventilation (BiPAP) is safe and feasible for hospitalized children with sickle cell disease (SCD). This supportive care may help prevent acute chest syndrome (ACS) in at-risk children.
Area of Science:
- Pediatric Hematology
- Pulmonology
- Critical Care Medicine
Background:
- Acute chest syndrome (ACS) is a significant cause of illness and death in children with sickle cell disease (SCD).
- Preventing hypoxemia by optimizing lung aeration during sleep is a clinical challenge in these patients.
Purpose of the Study:
- To evaluate the safety, feasibility, and tolerability of noninvasive bi-level positive airway pressure ventilation (BiPAP).
- To assess BiPAP as preventative, supportive care for hospitalized, medically stable children with SCD on a general pediatric unit.
Main Methods:
- Retrospective chart review of patients aged 22 years or younger with SCD.
- Inclusion criteria: admitted to a general pediatric unit, BiPAP recommended as supportive care.
- Exclusion criteria: PICU admission, BiPAP for respiratory failure, or home BiPAP use for OSA.
Main Results:
- BiPAP was recommended in 53 hospitalizations across 23 patients; 98% involved acute SCD pain.
- Indications included prior ACS (94%), chest/back pain (79%), and oxygen desaturation (66%).
- BiPAP was successfully used in 75% of hospitalizations for a median of two nights with no adverse effects. 88% of at-risk children tolerated BiPAP and did not develop ACS.
Conclusions:
- BiPAP is a safe, feasible, and well-tolerated supportive care option for hospitalized children with SCD.
- Further intervention trials are warranted to confirm BiPAP's efficacy in preventing ACS.
Background:
Acute chest syndrome (ACS) is a leading cause of morbidity and mortality among children with sickle cell disease (SCD). Preventing hypoxemia by optimizing lung aeration during sleep remains a challenge.
Objectives:
To explore safety, feasibility, and tolerability of noninvasive, bi-level positive airway pressure ventilation (BiPAP) as preventative, supportive care for hospitalized, medically stable children with SCD on a general pediatric inpatient unit.
Methods:
Retrospective chart review of patients ≤22 years of age with SCD admitted to the general pediatric inpatient unit from February 1, 2017 to March 1, 2020 for whom BiPAP was recommended as supportive care. Hospitalizations were excluded if patients were admitted to the pediatric intensive care unit (PICU), required BiPAP for respiratory failure, or used BiPAP at home for obstructive sleep apnea.
Results:
Twenty-three patients had 53 hospitalizations in which BiPAP was recommended. Fifty-two (98%) hospitalizations included acute SCD pain. Indications for BiPAP included prior ACS (94%), chest or back pain (79%), and/or oxygen desaturation (66%). On 17 occasions, patients already had mild to moderate ACS but were stable when BiPAP was recommended. BiPAP was used successfully during 75% of hospitalizations for a median of two nights. There were no adverse effects associated with BiPAP. PICU transfer for respiratory support occurred during three hospitalizations. In 26 hospitalizations of children at risk for ACS who tolerated BiPAP, 23 (88%) did not develop ACS.
Conclusions:
BiPAP is safe, feasible, and well tolerated as supportive care for hospitalized children with SCD. Next steps include an intervention trial to further assess the efficacy of BiPAP on ACS prevention.
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