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 Table of Contents  
ORIGINAL ARTICLE
Year : 2020  |  Volume : 11  |  Issue : 2  |  Page : 68-73

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


1 Prince Sultan Oncology Center, King Salman Armed Forces Hospital, KSA
2 Pediatric Intensive Care Unit, King Salman Armed Forces Hospital, KSA
3 Pediatric Department, King Salman Armed Forces Hospital, KSA
4 Pediatric Department, Faculty of Medicine, Tabuk University, KSA

Date of Submission20-Dec-2019
Date of Decision16-Mar-2020
Date of Acceptance03-Apr-2020
Date of Web Publication28-Jul-2020

Correspondence Address:
Dr. Ehab Hanafy
Prince Sultan Oncology Center, King Salman Armed Forces Hospital
KSA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/joah.joah_89_19

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  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.

Keywords: Acute chest syndrome, pediatric intensive care unit, Saudi Arabia, sickle cell disease, stroke, Tabuk


How to cite this article:
Hanafy E, Altoonisi M, Alghuraydh A, Alatawi A, Alsabah B, Alzahrani S, Attili I, Mahmoud G. Characteristics and outcomes of patients with sickle cell disease admitted to pediatric intensive care: A retrospective review. J Appl Hematol 2020;11:68-73

How to cite this URL:
Hanafy E, Altoonisi M, Alghuraydh A, Alatawi A, Alsabah B, Alzahrani S, Attili I, Mahmoud G. Characteristics and outcomes of patients with sickle cell disease admitted to pediatric intensive care: A retrospective review. J Appl Hematol [serial online] 2020 [cited 2020 Aug 6];11:68-73. Available from: http://www.jahjournal.org/text.asp?2020/11/2/68/290970


  Introduction Top


Sickle cell disease (SCD) is an autosomal recessive hematologic disorder that is common in the Kingdom of Saudi Arabia (KSA) with variable severity across the country.[1] Children with vaso-occlusive crisis (VOC) account for the majority of ward admissions, while acute chest syndrome (ACS) is a serious complication that is associated with a high risk of sickle cell-related morbidity and mortality, including pediatric intensive care unit (PICU) admissions.[2],[3] The most common cause for admission of SCD patients to intensive cares is ACS as reported in some studies.[4],[5] Nevertheless, VOC, which has an unpredictable course, should be carefully managed as it can progress easily to morbidities that justify intensive care unit (ICU) admission.[6] In addition to admitting patients with serious complications, we prefer to electively admit a proportion of stable SCD patients, including postoperative admissions and patients undergoing procedures such as exchange transfusion as a preventive intervention for stroke. Comprehensive health care and the use of disease-modifying agents in addition to blood transfusion programs have significantly improved the disease outcome. Our objectives in this review is to highlight the characteristic features of patients with SCD admitted to the PICU and who represent a single-hospital experience, also, to demonstrate the reason and type of admissions, different interventions done at the PICU, and to compare blood values for different admission groups and whether there are any associations with length of stay (LOS), and finally, to show the outcomes and mortality rate of SCD patients in the PICU.


  Methods Top


We retrospectively reviewed the medical records of pediatric patients <14 years of age with confirmed diagnosis of SCD by hemoglobin electrophoresis and who were admitted to the PICU over 6-year-period from December 2013 to December 2019. The study was done in King Salman Armed Forces Hospital (KSAFH), which is located in Tabuk, serving as a tertiary hospital and referral center for the northwestern region of KSA. The study was approved by the research and ethics committee in KSAFH. Data were collected using patient's files, electronic records, and PICU admission book. Patient's demographic characteristics were retrieved, in addition to previous medical history, laboratory values at admission, reason for PICU admission, LOS, interventions during PICU stay and status on discharge. All data were entered into Statistical Product and Service Solutions (SPSS v.20). The descriptive analysis of data, frequencies, and percentages are presented in tabulated and graphical form. Chi-square and Fisher's exact test are used to compare categorical variables, while t-test is used for comparing numerical variables. Pearson correlation coefficient is used to show associations between variables. P < 0.05 is considered statistically significant.

Definitions

Overt stroke

Acute neurologic insult caused by vascular occlusion or hemorrhages that result in ischemia and focal neurologic symptoms or signs lasting more than 24 h.[7]

Silent stroke

A magnetic resonance imaging evidence of cerebral ischemia in the absence of focal neurologic deficits.[7]

Transient ischemic attacks

A transient episode of neurologic dysfunction caused by focal brain, spinal cord, or retinal ischemia without acute infarction.[8]

Transcranial Doppler

Normal transcranial Doppler (TCD) is time-averaged maximum mean (TAMM) velocities <170 cm/sec, conditional TCD is TAMM velocities 170–199 cm/s, abnormal TCD is TAMM velocities ≥200 cm/s.[9]


  Results Top


The average number of active pediatric SCD patients <14 years following in our hospital per year is 240 patients. We identified 58 SCD patients (median age, 9 years; 33 males) [Figure 1], who were admitted 93 times to the PICU, accounting for 4.5% of total PICU admission over a 6-year-period from December 2013 to December 2019. This made up a rate of 6.5 admissions per 100 active SCD patients/year. Sixteen out of 58 patients were admitted more than once (2–6 times) for 51 admissions (21 elective). The majority of admissions were from the station (40.8%) and emergency room (ER) (35.4%), while the remaining admissions were from the hematology clinic and operating room [Figure 2]. We categorized the admissions into emergency and elective admissions (59 and 34 admissions, respectively) [Table 1].
Figure 1: Gender and age group for sickle cell disease patients admitted to the pediatric intensive care unit from December 2013 to December 2019

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Figure 2: Site and type of admission for sickle cell disease patients admitted to the pediatric intensive care unit from December 2013 to December 2019

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Table 1: Specifications of emergency and elective pediatric intensive care unit admissions for sickle cell disease patients

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Emergency admissions

ACS (n = 28) represented 30% of total admissions and the majority (47.5%) of emergency admissions; in addition, patients with ACS were admitted first to the station suffering VOC or mild-moderate respiratory manifestations before developing severe ACS that necessitated transfer to the PICU in 20 (71.4%) admissions. As a further matter, in a significant percentage of admissions, patients with ACS had high white blood cell (WBC) count and high hemoglobin S (HBS) level >50% (89% and 93% respectively). CVA was the second most common cause of admission to the PICU and constituted 18.2% of total admissions. Most of which were from the ER (64%), and in more than 70% of admissions, patients with CVA had high WBC count and high HBS level >70%. Five patients (5.37%) were admitted due to other neurological causes (2 transient ischemic attacks [TIAs], PRES,[10] acute soft head,[11] and subarachnoid hemorrhage [SAH]). Four admissions were due to gastrointestinal complications, while two patients were admitted in shock following splenic sequestration and 1 admission for each of sepsis, priapism, and ceftriaxone hypersensitivity.

Elective admissions

On the other hand, eight patients with the previous stroke were admitted electively 25 times (26.8% of total admissions) to do exchange transfusion as a part of stroke prevention program; 75% of these admissions were from clinic and station. While 8 patients were admitted 9 times (9.6%) for postoperative recovery following splenectomy, cholecystectomy, or cranial surgery. One of whom had splenectomy and cholecystectomy on 2 separate occasions.

A considerable number of exchange transfusion was performed for patients in 65 (69.8%) admissions; there was no significant association between exchange transfusion and admission categories, P = 0.56. While patients were more likely to have a simple blood transfusion in the emergency admissions (n = 16, 17.2%) than patients who were electively admitted, P < 0.05. Only three patients needed inotropic support and five patients needed invasive ventilation; however, oxygen support was required for patients in 43 (46.2%) admissions. Intravenous antibiotics were prescribed to patients in 65% of the PICU stay days, which was statistically significant in the emergency admissions group, P < 0.05. Furthermore, a central venous line was inserted to patients in 81 (87%) admissions, two patients were intubated, one patient had a chest tube inserted, and TCD was performed to one patient.

By comparing blood works in both emergency and elective admission groups, we found that WBC counts were significantly higher in the emergency group (mean, 18.37 × 103/μl) than in the elective group (mean, 10.4 × 103/μl), P < 0.05. Whereas, hemoglobin level showed statistically significant lower values in the emergency group (mean, 7.8 g/dl) than in elective group (mean, 9.67 g/dl), P < 0.05. Contrarily, mean platelet counts were 383 × 103/μl in the emergency group, and 347 × 103/μl in the elective group, which was not statistically significant, P = 0.36 [Table 2]. Moreover, patients had high HBS level more than 50% in 90% of the emergency admissions and in 58% of admissions in elective group. C-reactive protein (CRP) level was done for patients in 35 emergency admissions and high levels were detected in 27 (77%), and despite done only in 8 elective admissions, none of which had high CRP levels.
Table 2: Blood values for emergency and electively admitted sickle cell disease patients to the pediatric intensive care unit

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With regard to the 58 SCD patients admitted to the PICU (52 homozygous sickle cell anemia (HBSS), 6 HBS-β-thalassemia), 18 patients (31%) had previous PICU admissions, 13 patients (22.4%) had the previous stroke, 20 patients (34.4%) had previous ACS, whereas a significant number of patients (n = 37, 63.8%) had previous frequent admissions because of VOC. Approximately 27% of patients were on regular blood transfusion program, and 70% have been prescribed hydroxyurea prior to admission [Table 3].
Table 3: Characteristics of sickle cell disease patients admitted to the pediatric intensive care unit

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Finally, LOS in the PICU ranged from 1 to 14 days, with an average of 1.9 days, though patients stayed one day only in the majority of admissions (72%). LOS was positively correlated with WBC and CRP level, r (91) = 0.46, P = 0.00001 and r (41) = 0.4, P = 0.007, respectively, and showed negative correlation with Hb level and platelets count, r (91) = −0.26, P = 0.009 and r (91) = −0.25, P = 0.01, respectively. The mortality rate of SCD patients in PICU was low (n = 2, 3.4%), one patient (4-month old) presented with sepsis, and the other (5-year old) developed ceftriaxone hypersensitivity associated with severe hemolysis. Both patients had expired despite inotropes, invasive ventilation, and other supportive measures.


  Discussion Top


SCD is a common autosomal recessive hematologic disorder in Saudi Arabia. The prevalence of sickle cell trait ranges from 2% to 27%, and about 2.6% have SCD in some regions.[1] Manifestations of SCD are variable and include VOC, hemolysis, acute and chronic inflammations, which may end up with organ damage. ACS and cerebrovascular attacks can be severe enough to necessitate PICU admission.[12]

There is a paucity of data regarding the pediatric SCD patients admitted to the PICU. Only one study has been performed in the UK in this matter,[5] and our study is the first in Saudi Arabia and middle-east to reveal the characteristic features and outcomes for those cohort of patients who were admitted to the PICU.

Our study showed a mortality rate of (3.4%) which is relatively lower than what had been shown in Bartram et al.'s study (6%),[5] and even lower compared to results from studies in the UK and Oman that reported mortality rates of (19% and 16%) respectively[13],[14] in adult SCD population admitted to the ICUs. The relatively low mortality rate can be attributed to the younger population in our study, besides the early detection of complications that needs urgent PICU admission. Moreover, we prefer to electively admit a proportion of SCD patients to PICU, including postoperative admissions and patients undergoing procedures such as exchange transfusion as preventive intervention for stroke. This will ultimately ensure that the best quality of care can be offered to those patients.

According to the reason for admissions, we categorized them into emergency and elective groups. The study showed a total of 59 emergency admissions of which ACS was the most common reason for admission accounting for 30%, and this is comparable to other studies from adult and pediatric sides.[5],[12],[13],[14] ACS in pediatric SCD patients occurs more commonly in the western regions of Saudi Arabia and is considered one of the most common causes of death in SCD.[15] None of the patients with ACS died in our review, and all of them received intravenous antibiotics including ceftriaxone, macrolides and vancomycin, two needed mechanical ventilation, one required a chest tube and all required O2 support but none required inotropes. About (57%) required exchange transfusion versus (39%) who had a simple blood transfusion.

Studies on the prevalence of stroke in Saudi Arabia are limited. Prevalence in the western and northwestern regions of (9.4% and 4.2%), respectively, have been reported by two different studies.[16],[17] One of the main reasons for admission to PICU in our study was CVA. We admitted patients with CVA under the emergency and elective admission categories (18.2% and 26.8%, respectively). Patients with previous overt stroke or those who need primary prevention of stroke as detected by TCD or proven to have silent strokes were admitted under the elective group to perform exchange transfusion in an attempt to maintain HBS level <30%. In the emergency group, SCD patients were presenting for the first time with signs and symptoms of stroke and were admitted immediately to the PICU for supportive measures and exchange transfusion, only four patients in this group required intravenous antibiotics, and there was no mortality in this group.

Neurological reasons other than stroke that required PICU emergency admission included two patients with TIAs, one patient with SAH who required ventilation, inotropes, and had one of the longest stays in PICU (8 days). Other reasons include a patient with posterior reversible encephalopathy syndrome (PRES) who presented with hypertension and transient loss of vision and another patient with acute soft head who presented with severe headache, both patients improved dramatically and have been published as rare associations with SCD.[10],[11]

Four patients were admitted because of gastrointestinal complications that are associated with SCD, two were admitted with hepatic sequestration and required exchange transfusion, one with liver failure and the last one with acute ischemic pancreatitis who showed significant improvement in short period of time after exchange transfusion.

One patient (4-month-old) presented with severe sepsis and the other (5-year-old) admitted for fever and developed ceftriaxone hypersensitivity associated with severe hemolysis and one of the lowest hemoglobin levels that was recorded in our hospital (0.3 g/dl). Both patients had expired despite inotropes, invasive ventilation, intravenous antibiotics, and other supportive measures.

Out of the 34 elective admissions, eight patients were admitted 9 times (9.6%) for postoperative recovery following splenectomy, cholecystectomy, or cranial surgery to resolve craniosynostosis. One of whom had splenectomy and cholecystectomy on two separate occasions. The postoperative admission to PICU for full recovery before transfer to the station is considered one of the most important improvements done in our hospital to ensure the best of care to pediatric SCD patients.

Central venous lines are one of the most common procedures performed in PICU; in our review, patients had central line inserted in 81 (87%) admissions, and this was attributed mainly to the high number of exchange transfusion that has been done in the PICU for those patients. There were no reported complications from central venous lines during the PICU stay.

Some studies for adult SCD population correlate blood values to the mortality rates in intensive care. Shome et al. stated that platelet counts correlate inversely with prognostic scores (Acute Physiology and Chronic Health Evaluation II and multiple organ dysfunction scores) and are significantly reduced in multi-organ failure and nonsurvivors.[18] Other study showed that there was no statistical significance in the hemoglobin level and HBS level for both survivors and nonsurvivors SCD patients in the intensive care.[14]

However, due to the lower mortality rate in our review, we preferred to highlight the difference in blood values between the emergency and elective admission instead, where WBC count and hemoglobin level showed significant difference between the two groups P < 0.05, while platelet counts showed no difference P = 0.36. Average LOS in the PICU was 1.9 days which is comparable to the study done in the UK for the pediatric SCD,[5] but shorter than studies done for adult SCD population, which ranged from a median of 3–6 days.[12],[13] LOS had shown also a positive correlation with WBC count and CRP levels but was negatively correlated to hemoglobin and platelet counts. Other studies are needed to demonstrate possible laboratory and clinical variables that might predict the admission of pediatric SCD patients to the PICU.

The medical history for the 58 patients admitted to the PICU showed that 31% of patients had previous PICU admissions, 22.4% had previous stroke, 34.4% had previous ACS, 63.8% had previous frequent admissions because of VOC, and 27% of patients were on regular blood transfusion program.

Seventy percent of patients have been prescribed hydroxyurea prior to admission, though the exact compliance could not be determined; however, it was noted that mean corpuscular volume was 78.8 fL in emergency group and 84.8 fL in elective group which are low values for patients supposed to be compliant to hydroxyurea. Similarly, low values of HB F level were detected for both emergency and elective groups (mean, 9.9% and 11.8% respectively).


  Conclusion Top


This is the first study in KSA and the middle east to reveal the characteristic features and outcomes of pediatric SCD patients admitted to the intensive care. PICU plays a pivotal rule in the management of patients with SCD. Outcomes of this cohort of patients have dramatically improved in the presence of highly dedicated professional team, who are undoubtedly essential participants in the comprehensive health-care services presented to SCD patients.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Bartram JL, Thein SL, Gardner K, Egberongbe Y, D'Silva P, Height SE, et al. Outcome of children with sickle cell disease admitted to intensive care – A single institution experience. Br J Haematol 2010;150:614-7.  Back to cited text no. 5
    
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Sacco RL, Kasner SE, Broderick JP, Caplan LR, Connors JJ, Culebras A, et al. An updated definition of stroke for the 21st century: A statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2013;44:2064-89.  Back to cited text no. 7
    
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Hanafy E, Alshareef D, Osman S, Al Jabri A, Nazim F, Mahmoud G. Posterior reversible encephalopathy syndrome secondary to asymptomatic poststreptococcal glomerulonephritis in a child with sickle cell anemia: A case report. J Med Case Rep 2018;12:24.  Back to cited text no. 10
    
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[PUBMED]  [Full text]  


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