Non Invasive Positive Pressure Ventilation for Prevention of Acute Chest Syndrome in Sickle Cell Disease
Non Invasive Positive Pressure Ventilation for Prevention of Acute Chest Syndrome in Sickle Cell Disease
Acute chest syndrome is a severe respiratory complication of sickle cell disease.

 The standard prevention of this dangerous complication is spirometry in wich patient is
 required to take deep breaths trough a little device several times a day. This treatment is
 compromised when pain in important or when the patient is asleep and cannot participate.

 The investigators hypothesised that non invasive ventilation in wich patient have no effort
 to take might be a better prevention than spirometry and may improve pain and quality of
 sleep.

 Children with vaso-occlusive crisis necessitating morphinic treatment will be randomly
 assigned with either spirometry or ventilation and the investigators will monitor for
 occurrence of acute chest syndrome, pain and quality of sleep.
Acute chest syndrome is a severe complication of sickle cell disease. Several factors can
 contribute to this complication including infections, vaso-occlusive crisis, important pain
 that may give shortness of breath and morphinic treatments as they may cause hypoventilation.

 The standard prevention of this dangerous complication is incentive spirometry in wich
 patient is required to take deep breaths trough a little device several times a day. This
 treatment is compromised when pain in important or when the patient is asleep and cannot
 participate.

 In patients who have had surgery who have also pain and morphinic treatments, non invasive
 ventilation have proven a good option for preventing pulmonary complications.

 Besides, our experience in non invasive ventilation for treatment of acute chest syndrome
 show a good tolerance and efficiency. It also seems to help with pain management.

 The investigators hypothesised that non invasive ventilation in wich patient have no effort
 to take might be a better prevention than incentive spirometry and may improve pain and
 quality of sleep.

 After informed consent, patients aged from 6 to 20 years old with SS or S-Beta sickle cell
 disease with vaso-occlusive crisis necessitating morphinic treatment hospitalised in
 intensive care unit in Necker Hospital for Sick Children (Paris) will be randomly assigned
 with either spirometry or ventilation. It is a monocentric study.

 Patients with acute chest syndrome, oxygen needs, pneumonia or other condition requiring
 ventilation at presentation will not be included.

 Spirometry will be realised through volumetric devices, every two hours during the day with
 10 maximum inspirations and at night if the patient is awake.

 Non invasive ventilation will be applied through a nasal mask with VS III machines from
 Resmed®, at least one hour every four hour but may be maintained more if the patient chooses
 to or is asleep with the machine on.

 The investigators will monitor for occurrence of acute chest syndrome, pain, morphinic
 requirements, length of hospital stay, comfort, quality of sleep The treatment will be
 continued until morphinic treatment is stopped. The investigators intend to include 60
 patients in each arm to show a difference of 25% in the occurrence of acute chest syndrome
 (primary judgement criteria).

 Acute chest syndrome will be defined according to the criteria used by Vichinsky (Vichinsky,
 E. P. et al. Acute chest syndrome in sickle cell disease: clinical presentation and course.
 Cooperative Study of Sickle Cell Disease. Blood 89, 1787-1792 (1997) The investigators will
 aslo compare morphinic requirements, length of hospital stay and quality of sleep as
 secondary judgment criteria.
Inclusion Criteria:

 -  Patient with SS or S-Beta sickle cell disease aged more than 6 years old and less then
 20 years old

 -  Hospitalised since less then 24 hours for severe vaso-occlusive crisis (either
 peripheric localisation or thoracic crisis) with morphinic requirements according to
 local pain management protocol

 -  With a signed informed consent from the patient or his legal representative if the
 patient is under 18 years old

 -  Benefiting of the French social security system

 Exclusion Criteria:

 -  Patient that has already participated in the study

 -  Oxygen requirements, pneumonia, acute chest syndrome or other condition necessitating
 non invasive ventilation on admission

 -  No understanding of spirometry or ventilation techniques

 -  Other condition that forbid the use of non invasive ventilation or spirometry as
 defined by french consensus conference (2006) on non invasive ventilation