Sleep Respiratory Disorders Evaluation in Sickle Cell Disease Children

Study Overview
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Brief Summary
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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.

Detailed Description
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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.

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

Conditions
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Sickle Cell Disease, Respiration Disorders

Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 2 Years
Maximum Age: 6 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Vaso-occlusive and cerebral complications frequency | Vaso-occlusive complications frequency and abnormal transcranial doppler (TCD) during the 18 months of follow up (cohort study) 3 subpopulations will be identified upon polysomnographic results: Group 1: normal polysomnography Group 2: sleep abnormalities related to obstructive upper airway disease or bronchial disease Group 3: sleep abnormalities associated with non-obstructive pattern and isolated hypoxemia identified. The therapeutic trial will be done on this group. | 18 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Respiratory sleep abnormalities frequency | Respiratory sleep abnormalities frequency in a population of young sickle cell disease children. | 18 months | 
| Relationship between sleep abnormalities and transcranial doppler abnormalities | Analyze the relationship between sleep respiratory and non-respiratory abnormalities and transcranial doppler abnormalities | 2 months | 
| Polysomnography relevance compared to sleep ventilatory polygraphy | Polysomnography relevance compared to sleep ventilatory polygraphy in diagnosis of sleep abnormalities | 2 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Sickle Cell Disease, Respiration Disorders, Polysomnography