Supportive Noninvasive Ventilation Safe, Tolerated in Sickle Cell Children


More studies are needed to determine whether bi-level positive airway pressure ventilation can help prevent acute chest syndrome.

sickle cell

Cara Guenther, MD

Findings from a new study support the safety and tolerability of bi-level positive airway pressure ventilation (BiPAP) as supportive care for hospitalized children with sickle cell disease.

“While incentive spirometry has been shown to be effective at preventing acute chest syndrome (ACS) in hospitalized children with acute SCD pain, this intervention can only be used during waking hours,” noted the study investigators. “An additional strategy is needed to prevent oxygen desaturation and hypoventilation for these patients during sleep.”

BiPAP was first used as supportive care in the Boston Medical Center pediatric inpatient unit in February of 2017. To help with breathing, the device offers non-invasive ventilation through a mask and provides positive airway pressure during exhalation in addition to higher pressure breaths during inhalation.

A team, led by Cara Guenther, MD, Boston Medical center, conducted a retrospective chart review where BiPAP was recommended as supportive care for patients with sickle cell disease and ≤22 years of age.

All patients were admitted in the unit between February 1, 2017-March 1, 2020.

Guenther and colleagues primarily evaluated the proportion of hospitalizations in which BiPAP was used successfully for ≥1 night. Secondary outcomes included tolerability, adverse events attributable to BiPAP, and development of ACS or worsening respiratory distress requiring care in the intensive care unit.

Overall, they evaluated 23 patients representing 53 hospitalizations. Median age at time of admission was 16 years old.

All had hemoglobin SS, 20 were on hydroxyurea, and 20 had a history of acute chest syndrome — ACS was diagnosed at admission in 15 hospitalizations.

Furthermore, 11 patients had 1 or more ICU admission for respiratory issues.

“Indications for BiPAP included prior ACS (n = 50, 94%), chest/back pain (n = 42, 79%), oxygen desaturation (n = 35, 66%), his- tory of an abnormal sleep study (n = 15, 28%), and/or mild to moderate ACS diagnosed at or after admission but not requiring PICU-level care (n = 20, 38%),” the investigators reported.

Of the 53 hospitalizations, BiPAP was used in 40 hospitalizations for a median of 2 nights. For some patients, BiPAP was not initially tolerated but tolerated in subsequent attempts.

The mask proved to be a common reason for inability to tolerate the BiPAP. Also, some patients found it difficult to breath with the positive pressure.

Over the course of the study period, BiPAP utilization increased from use in 12 hospitalizations in 2017 to 24 hospitalizations in 2019. Further, there were no adverse events associated with BiPAP use.

ACS was diagnosed in 17 hospitalizations in the emergency department. A patient developed suspected ACS one day following BiPAP use.

ICU transfer for respiratory support occurred in 3 hospitalizations, 2 of which occurred in the same patient (who had a history of severe sickle cell disease and recurrent ACS).

And finally, among 26 hospitalizations in which high-risk patients had no history of ACS, 23 did not progress to ACS after BiPAP use.

“We have demonstrated that using BiPAP on a general pediatric inpatient unit is safe, feasible, and well tolerated as supportive care for hospitalized children with SCD at risk for adverse respiratory outcomes,” Guenther and team wrote.

However, they indicated more data is needed to ascertain the efficacy of BiPAP for treatment and prevention of ACS in a pediatric ICU setting.

The study, “SNAP: Supportive noninvasive ventilation for acute chest syndrome prevention in children with sickle cell disease,” was published online in Pediatric Blood Cancer.

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