Predictors of Mortality in Adults With Sickle Cell Disease Admitted to the Intensive Care Unit in King Saud Medical City, Saudi Arabia

Background Sickle cell disease (SCD) is the most common genetic blood disorder in Saudi Arabia. A limited number of studies have been conducted on SCD patients regarding their intensive care unit (ICU) admissions. We aimed to identify the cause of ICU admission in SCD patients and to identify predictors of mortality. Methodology We identified 64 patients with SCD, aged 14 years and older, who were admitted to the ICU of King Saud Medical City, Riyadh, Kingdom of Saudi Arabia, from January 1, 2017, to December 31, 2020. Results Acute chest syndrome was the most frequent primary diagnosis for ICU admission in 29 (45.3%) patients followed by vaso-occlusive crisis in 23 (35.9%) patients. Pregnancy in eight (12.5%) patients was the most prevalent co-existing condition. The median age was 29 years, with males comprising 45.3% and females comprising 54.7% of the study population. Arterial blood gas pH less than 7.2 on ICU admission (p= <0.001), hemodialysis support (p= 0.049), the use of vasopressors (p= 0.016), intubation (p= <0.001), and being intubated within the first 24 hours of ICU stay (p= 0.04) had a statistically significant association with mortality at ICU discharge out of all the variables tested. Mortality on ICU discharge was 7 (10.9%). Conclusion This was a retrospective study carried out in King Saud Medical City. Comparing the results of the study to those of similar ones conducted around the world revealed a low SCD ICU mortality rate. This low mortality may be a result of improved overall ICU care. We recommend a multi-center, prospective study in future.

[1]  J. Reignier,et al.  Nationwide retrospective study of critically ill adults with sickle cell disease in France , 2021, Scientific Reports.

[2]  Sana Abdulaziz Al Khawaja,et al.  Predictors of mortality in adults with Sickle cell disease admitted to intensive care unit in Bahrain , 2017, Journal of critical care.

[3]  R. Ware,et al.  Sickle cell disease , 2017, The Lancet.

[4]  A. Douiri,et al.  Survival in adults with sickle cell disease in a high-income setting. , 2016, Blood.

[5]  F. Piel,et al.  Environmental determinants of severity in sickle cell disease , 2015, Haematologica.

[6]  A. Ashley-Koch,et al.  Factors associated with survival in a contemporary adult sickle cell disease cohort , 2014, American journal of hematology.

[7]  P. Frenette,et al.  Vaso-occlusion in sickle cell disease: pathophysiology and novel targeted therapies. , 2013, Hematology. American Society of Hematology. Education Program.

[8]  Tamoghna Biswas Global burden of sickle cell anaemia is set to rise by a third by 2050 , 2013, BMJ.

[9]  Qutaiba A. Tawfic,et al.  Adult Sickle Cell Disease: A Five-year Experience of Intensive Care Management in a University Hospital in Oman. , 2012, Sultan Qaboos University medical journal.

[10]  Z. Memish,et al.  Six-year outcome of the national premarital screening and genetic counseling program for sickle cell disease and β-thalassemia in Saudi Arabia , 2011, Annals of Saudi medicine.

[11]  W. Jastaniah Epidemiology of sickle cell disease in Saudi Arabia , 2011, Annals of Saudi medicine.

[12]  J. Elion,et al.  [Pathophysiology of sickle cell disease]. , 2010, Medecine tropicale : revue du Corps de sante colonial.

[13]  S. Thein,et al.  Outcome of adults with sickle cell disease admitted to critical care – experience of a single institution in the UK , 2010, British journal of haematology.

[14]  Cage S. Johnson,et al.  Outcome of Sickle Cell Anemia: A 4-Decade Observational Study of 1056 Patients , 2005, Medicine.

[15]  Arvind K. Shah,et al.  Causes of death in sickle cell disease: an autopsy study , 2003, British journal of haematology.

[16]  R. Hebbel,et al.  The molecular pathobiology of cell membrane iron: the sickle red cell as a model. , 1998, Free radical biology & medicine.

[17]  M. Steinberg 6 Pathophysiology of sickle cell disease , 1998 .

[18]  P. Swerdlow,et al.  Clinical Characteristics and Outcome of Patients with Sickle Cell Disease Admitted to the Medical Intensive Care Unit , 2017 .

[19]  F. Roudot-thoraval,et al.  Patterns of mortality in sickle cell disease in adults in France and England. , 2002, The hematology journal : the official journal of the European Haematology Association.