Sleep Respiratory Disorders Evaluation in Sickle Cell Disease Children

Systematic Evaluation of Sleep Respiratory Disorders During Sleep in Children With Sickle Cell Disease

In Sickle cell disease children, sleep respiratory abnormalities are risk factors for vaso-occlusive complications, as well as cerebral vasculopathy.

A 18 months follow-up children with sickle cell disease evaluating sleep respiratory problems frequency and etiology, as well as their influence on sickle cell disease complications.

• Inclusion visit with physical examination. A 2.9 ml blood sample will be necessary, if not done within the framework of care within 6 months for: CBC (Cell Blood Count), reticulocytes counts (1.2 ml of blood), liver enzymes, electrolytes, urea, creatininemia (1,2 ml of blood) and fetal hemoglobin (0.5 ml of blood). - A complete standardized examination including nasopharynx endoscopy, if not yet done in usual care, to identified upper airway obstruction
• A standardized pneumology evaluation to identified obstructive lung symptomatology
• An ambulatory polysomnography will be performed within days following inclusion, during sleep and will allow electroencephalogram recording, oculomotricity, muscles (mentalis muscles and tibialis anterior) movements, electrocardiogram and respiratory activity recording.

The steering committee will classify the children in three populations based on their exam results:

• Group 1: no abnormality in polysomnography
• Group 2: obstructive sleep-related disorder
• Group 3: isolated sleep hypoxemia for group3, patients will have a new ambulatory polysomnography.

Inclusion Criteria:

• Sickle cell disease children with either sickle cell anemia, compound heterozygozity SB0-thalassemia, or SDPunjab,
• no prior transfusion program within 3 months,
• no prior treatment with hydroxycarbamide within 3 months,
• Aged 2 to 6 years,
• Parents or tutors signed informed consent,
• Prior physical examination,
• Social security insurance.

Exclusion criteria:

• Encephalopathy
• Other disease that could interfere with protocol exams realization
• Nitrous oxide analgesia contre-indication
• Conventional hospitalization in the past 3 weeks