Characteristics and outcomes of patients with sickle cell disease admitted to pediatric intensive care: A retrospective review

Abstract

INTRODUCTION: Sickle cell disease (SCD) is autosomal recessive disorder common in the Kingdom of Saudi Arabia (KSA). This is first study in KSA, and middle-east revealing characteristics of pediatric SCD patients admitted to pediatric intensive care unit (PICU). METHODS: Retrospective review of SCD patients admitted to PICU over 6-years-period (2013-2019). Data collected using patient's files and PICU book. Descriptive analysis and percentages presented in tabulated and graphical form. RESULTS: Fifty-eight patients admitted 93 times to PICU, (4.5% of total PICU admission). Most admissions were from station and emergency room (38, 33 admissions respectively). Admissions categorized into emergency and elective (59, 34 admissions respectively). Average length of stay was 1.9 days. Acute chest syndrome (ACS) (n = 28) represented 30% of admissions, followed by cerebrovascular accident (CVA) (18.2%). Five patients (5.37%) admitted for other neurological causes. Twenty-five (26.8%) admitted electively for stroke prevention, 9 (9.6%) admitted postoperatively. Considerable number of exchange transfusion in 65 (69.8%) admissions. Three needed inotropes, five invasive ventilation. Thirteen/58 (22.4%) had previous stroke, 20 (34.4%) had previous ACS, whereas significant number (n = 37, 63.8%) had frequent vaso-occlusive crisis. Mean white blood cell was 18.37 × 103/μl (emergency group), and 10.4 × 103/μl (elective group). Mean hemoglobin 7.8 g/dl and 9.67 g/dl in emergency and elective groups, respectively. Seventy percent of patients prescribed hydroxyurea and 27% of patients were on regular transfusion. Mortality rate of SCD in PICU was low (n = 2, 3.4%). CONCLUSION: PICU is one of the essential components of comprehensive health-care services for patients with SCD. Cooperation between intensivists, hematologists, and other health-care providers in addition to the early referral of critically ill SCD patients to PICU leads to better disease outcomes.