Nitric Oxide Therapy for Acute Chest Syndrome in Sickle Cell Disease Children (INNOSTAPED)

• Number of blood transfusions and total transfused blood volume [ Time Frame: 7 to 10 days ] [ Designated as safety issue: No ]
• Quantity of Pain-killer drugs required and particularly OPIOIDS [ Time Frame: 7 to 10 days ] [ Designated as safety issue: No ]
• Duration of Nitric oxide therapy [ Time Frame: after 7 to 10 days ] [ Designated as safety issue: No ]
• Duration of OXYGENOTHERAPY [ Time Frame: 7 to 10 days ] [ Designated as safety issue: No ]

• Number of blood transfusions and total transfused blood volume [ Time Frame: 7 to 10 days ] [ Designated as safety issue: No ]
• Quantity of Pain-killer drugs required and particularly opioïds [ Time Frame: 7 to 10 days ] [ Designated as safety issue: No ]
• Duration of Nitric oxide therapy [ Time Frame: after 7 to 10 days ] [ Designated as safety issue: No ]
• Duration of OXYGENOTHERAPY [ Time Frame: 7 to 10 days ] [ Designated as safety issue: No ]

Acute chest syndrome is a severe sickle cell disease complication in children requiring blood transfusion therapy to prevent acute respiratory failure and death. Nitric oxide is a potent vasodilator that could reverse pulmonary vascular occlusion and restore normal oxygenation. The randomized trial will test that hypothesis.

Acute chest syndrome is a severe sickle cell disease complication in children requiring blood transfusion therapy to prevent acute respiratory failure and death. Nitric oxide is a potent vasodilator that could reverse pulmonary vascular occlusion and restore normal oxygenation. The randomized trial will test that hypothesis in a prospective randomized double-blind placebo controlled study. 50 children in two years will be included: 25 in each arm.

• Acute Chest Syndrome
• Sickle Cell Disease

• Drug: Nitric oxide by inhalation INOMAX

  Nitric oxide by inhalation INOMAX 800 ppm

  40 ppm during 24 hours then 20 ppm during 24 hours then 10 ppm during 24 hours

  Other Name: INOMAX
• Drug: Placebo
  placebo
  Other Name: Placebo

• Active Comparator: 1: INOMAX

  Nitric oxide by inhalation INOMAX:

  active arm treated with nitric oxide

  Intervention: Drug: Nitric oxide by inhalation INOMAX
• Placebo Comparator: 2: Placebo
  placebo arm treated with placebo at the same conditions
  Intervention: Drug: Placebo

Inclusion Criteria:

• child between 1 and 18 years old
• Sickle cell anemia or equivalent (sickle beta 0 thalassemia)whoe weight is between 10kg and 65kg
• presenting acute chest syndrome as defined a new radiological infiltrate with tachypnea, respiratory discomfort, cough, chest wall pain and fever more than 38.5°C
• hypoxaemia with transcutaneous oxygen saturation equal or less than 92%
• informed consent signed by parents and approved by the child able to express his consent
• insured by the National social security system or by the universal medical insurance
• previous medical physical examination

Exclusion Criteria:

• respiratory distress with hypoxaemia with transcutaneous oxygen saturation equal or less than 92% under more than 5l/min of oxygen or 40% oxygen inhaled, hypercapnia signs 'sweating, altered consciousness, paCO2 more than 60mmHg) with need of emergency exchange transfusion and/or tracheal intubation with mechanical ventilation
• Isolated acute asthmatic crisis
• stroke or priapism with emergency acute transfusion needed
• acute anemia with hemoglobin drop of more than 20% as compared to steady state hemoglobin
• chronic long term transfusion therapy
• nitric oxyde hypersensitivity
• patients with right-left extra-pulmonary cardiac shunt
• patient previously included in the protocol
• patient participating in another interventional protocol
• pregnancy or breast feeding