Is the Preoperative Preparation of Sickle Cell Patients Optimal: Assessment of Practices and Post-operative Complications

Study Overview
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Brief Summary
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Children with sickle cell disease systematically receive a transfusion 2 to 5 days before scheduled surgery (with the exception of minor surgeries) in order to avoid post-operative complications of which the vaso-occlusive crisis and acute thoracic syndrome are the most frequent. This standardized preoperative protocol was established on the basis of the results of large-scale randomized studies, most of which date back over ten years, and which have demonstrated the beneficial effects of transfusion (or transfusion exchange) preoperatively. To date, several other more recent studies (but not controlled) have questioned this type of systematic management. The purpose of this study is to review retrospectively data of sickle cell children who have undergone elective surgery at the Huderf in the last ten years and to identify the eventual complications encountered. The most common procedures in these patients are: tonsillectomy with or without associated adenoids, splenectomy and cholecystectomy. General data on sickle cell disease (history, genotype, G6PD deficiency, biology and previous complications), pre-surgical preparation, surgery and post surgical management and complications will be collected and analyzed. This retrospective analysis will allow an objective assessment of the current quality of care and will provide useful data to improve patient management.

Official Title
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Is the Preoperative Preparation of Sickle Cell Patients Optimal: Retrospective Assessment of Practices and Post-operative Complications in a Cohort of Children Followed at Hôpital Universitaire Des Enfants Reine Fabiola (HUDERF) and Who Have Been Managed According Local Guidelines Including Transfusion or Exchange Transfusion Before Surgery

Conditions
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Sickle Cell Disease

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Sickle cell disease with surgery during the 2010-2019 period Exclusion Criteria: None

Ages Eligible for Study
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Maximum Age: 18 Years

Sexes Eligible for Study
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All

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 |
| --- | --- | --- |
| To assess the overall incidence of vaso-occlusive-events 1 month post surgery | Vaso-occlusive-events will include Vaso Occlusive Crisis (acute pain events that requires a visit to a medical facility and administration of pain medications (opioids or IV NSAIDs)), acute chest syndrome (new pulmonary infiltrate associated with by pneumonia-like symptoms, pain or fever) and hemoglobin < 6g/dl | 1 month after surgery | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| To assess the incidence of Vaso Occlusive Crisis 1 month post surgery | Vaso Occlusive Crisis is defined as acute pain events that requires a visit to a medical facility and administration of pain medications (opioids or IV NSAIDs) | 1 month after surgery | 
| To assess the incidence of Vaso Occlusive Crisis 3 months post surgery | Vaso Occlusive Crisis is defined as acute pain events that requires a visit to a medical facility and administration of pain medications (opioids or IV NSAIDs) | 3 months after surgery | 
| To assess the incidence of Vaso Occlusive Crisis 12 months post surgery | Vaso Occlusive Crisis is defined as acute pain events that requires a visit to a medical facility and administration of pain medications (opioids or IV NSAIDs) | 12 months after surgery | 
| To assess the incidence of Acute Chest Syndrome 1 month post surgery | Acute Chest Syndrome is defined as a new pulmonary infiltrate associated with by pneumonia-like symptoms, pain or fever | 1 month after surgery | 
| To assess the incidence of Acute Chest Syndrome 3 months post surgery | Acute Chest Syndrome is defined as a new pulmonary infiltrate associated with by pneumonia-like symptoms, pain or fever | 3 months after surgery | 
| To assess the incidence of Acute Chest Syndrome 12 months post surgery | Acute Chest Syndrome is defined as a new pulmonary infiltrate associated with by pneumonia-like symptoms, pain or fever | 12 months after surgery | 
| To assess the duration of hospitalization post-surgery | number of days between surgery and day of discharge | 1 month after surgery | 
| To assess the incidence of Red Blood Cell (RBC) allo-immunization 1 month post surgery | incidence of acute anemia with the presence of new allo-antibody(ies) against RBC | 1 month after surgery | 
| To assess the incidence of RBC allo-immunization 3 months post surgery | incidence of acute anemia with the presence of new allo-antibody(ies) against RBC | 3 months after surgery | 
| To assess the incidence of RBC allo-immunization 12 months post surgery | incidence of acute anemia with the presence of new allo-antibody(ies) against RBC | 12 months after surgery | 
| To assess the number of RBC transfusion episodes 1 month post surgery | Number of RBC unit transfused | 1 month after surgery |