Sickle cell disease (SCD) is characterized by recurrent vaso-occlusive pain crisis (VOC),
which may evolve to acute chest syndrome (ACS), the most common cause of death among adult
patients with SCD. Currently, there is no safe and effective treatment to abort VOC or
prevent secondary ACS. Management of VOC mostly involve a symptomatic approach including
hydration, analgesics, transfusion, and incentive spirometry, which was investigated in a
very limited number of patients (<30).
The polymerisation of HbS is one major feature in the pathogenesis of vaso-occlusion. Among
factors determining the rate and extent of HbS polymer formation, the hypoxic stimulus is one
of the most potent and readily alterable. Current guidelines recommend oxygen therapy in
patients with VOC in order to maintain a target oxygen saturation of 95%. Low-flow nasal
oxygen (LFNO) is routinely used to achieve this normoxia approach, particularly in patients
at risk of secondary ACS because they may experience acute desaturation. In contrast, various
case series suggest a potential beneficial role of intensified oxygen therapy targeting
hyperoxia for the management of VOC, particularly with the use of hyperbaric oxygen, but the
latter is difficult to implement in routine clinical practice.
A recent high-flow nasal oxygen (HFNO) technology allows the delivery of humidified gas at
high fraction of inspired oxygen (FiO2) through nasal cannula. The FiO2 can be adjusted up to
100% (allowing hyperoxia that may reverse sickling) and the flow can be increased up to 60
L/min (which generates positive airway pressure and dead space flushing, that may prevent
evolution of VOC towards ACS by alleviating atelectasis and opioid-induced hypercapnia). In
patients with acute respiratory failure, HFNO has been shown to improve patient's comfort,
oxygenation, and survival as compared to standard oxygen or non-invasive ventilation.
The aim of the present study is to test the efficacy and safety of HFNO for the management of
VOC and prevention of secondary ACS. The investigators will use a multi-arm multi-stage
(MAMS) design to achieve these goals. HFNO will be delivered through AIRVO 2 (Fisher and
Paykel Healthcare, New Zealand), a device that incorporates a turbine allowing its use in
hospital wards.