Abstract
Sickle cell disease (SCD) is an inherited disease caused by an abnormal type of haemoglobin. It is one of the most common genetic blood disorders in the Gulf area, including Oman. It may be associated with complications requiring intensive care unit (ICU) admission. This study investigated the causes of ICU admission for SCD patients. This was a retrospective analysis of all adult patients ≥12 years old with SCD admitted to Sultan Qaboos University Hospital (SQUH) ICU between 1st January 2005 and 31st December 2009. A total number of 49 sickle cell patients were admitted 56 times to ICU. The reasons for admission were acute chest syndrome (69.6%), painful crises (16.1%), multi-organ failure (7.1%) and others (7.2%). The mortality for SCD patients in our ICU was 16.1%. The haemoglobin (Hb) and Hb S levels at time of ICU admission were studied as predictors of mortality and neither showed statistical significance by Student's t-test. The odds ratio, with 95% confidence intervals, was used to study other six organ supportive measures as predictors of mortality. The need for inotropic support and mechanical ventilation was a good predictor of mortality. While the need for non-invasive ventilation, haemofiltration, blood transfusions and exchange transfusions were not significant predictors of mortality. Acute chest syndrome is the main cause of ICU admission in SCD patient. Unlike other supportive measures, the use of inotropic support and/or mechanical ventilation is an indicator of high mortality rate SCD patient.
Highlights
Advances in knowledge - This study gives a descriptive analysis for sickle cell patients admitted to an intensive care unit
Sickle cell disease (SCD) is an autosomal recessive disease caused by an abnormal type of haemoglobin, termed haemoglobin S (Hb S).This haemoglobin is caused by the substitution of valine for glutamic acid at the sixth amino acid position of the beta chain.[1, 2]
This study was a descriptive analysis of sickle cell patients who were admitted with complications to the intensive care unit (ICU) at Sultan Qaboos University Hospital, Oman, over five years and investigated the predictive factors for sickle cell mortality
Summary
Advances in knowledge - This study gives a descriptive analysis for sickle cell patients admitted to an intensive care unit. The most common complications associated with SCD include vasoocclusive pain crises (VOC), acute chest syndrome, severe anaemia, infection, cerebral vascular accidents, and multiorgan failure.[4,5] Because of the fact that there has been little progress in fully understanding the pathophysiology of SCD and finding a cure for it, our management is primarily focusing on treating the negative sequelae and complications of the disease.[6,7] This study was a descriptive analysis of sickle cell patients who were admitted with complications to the ICU at Sultan Qaboos University Hospital, Oman, over five years and investigated the predictive factors for sickle cell mortality
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