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Year : 2021  |  Volume : 10  |  Issue : 1  |  Page : 38-42

The pattern of health care provision and utilization among adult patients with sickle cell disease in Southern Saudi Arabia

1 Department of Medicine, College of Medicine, King Khalid University, Abha, Saudi Arabia
2 College of Medicine, King Khalid University, Abha, Saudi Arabia
3 Department of Medicine, Aseer Central Hospital, Abha, Saudi Arabia
4 Department of Laboratory and Blood Bank, Aseer Central Hospital, Abha, Saudi Arabia

Date of Submission14-Oct-2020
Date of Decision29-Jan-2021
Date of Acceptance05-Feb-2021
Date of Web Publication02-Apr-2021

Correspondence Address:
Husain Y Alkhaldy
Department of Internal Medicine, College of Medicine, King Khalid University, Aseer Central Hospital, Abha
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/sjhs.sjhs_228_20

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Background: Sickle cell disease is a common hereditary hemoglobin disorder in southern Saudi Arabia. The current models of health care provision and health service utilization are not homogeneous across the kingdom. Aim: The current models of health care provision and health service utilization in a single center in our region were audited to evaluate the current provided healthcare services and suggest improvement if needed. Methodology: In this retrospective chart review study, from 2015 to 2019, all patients with sickle cell anemia admitted to a tertiary care hospital were enrolled in the study. All related data including demographics, laboratory interventions, and management procedures were collected. Descriptive analysis and statistical tests of association were performed. Results: One hundred and twenty-one patients were admitted 165 times over the study period. Vaso-occlusive pain episodes was the most commonindication (58%) for admission, followed by hepatobiliary (16%), and pulmonary complications (12.7%). Most of the provisioned services were of nonspecialized medical or surgical specialties. Blood transfusion was overused (64%), whereas adequate hydroxyurea (HU) therapy was underused (25%). Conclusion: The current study offers a glimpse of some gaps between the current practice and the established local, national, and international guidelines in terms of underutilizing HU and over utilizing blood transfusion. Comprehensive care centers were not available, and care was provided mainly by nonhematologists. Educating health providers and establishing comprehensive care centers would enhance the current model of health care provision.

Keywords: Health care, provision, sickle cell

How to cite this article:
Alkhaldy HY, Asiri BS, Ahmed MA, Khozam SA, Alzahrani AA, Alqahtani YM, Alshamrani ND, Bakheet OS. The pattern of health care provision and utilization among adult patients with sickle cell disease in Southern Saudi Arabia. Saudi J Health Sci 2021;10:38-42

How to cite this URL:
Alkhaldy HY, Asiri BS, Ahmed MA, Khozam SA, Alzahrani AA, Alqahtani YM, Alshamrani ND, Bakheet OS. The pattern of health care provision and utilization among adult patients with sickle cell disease in Southern Saudi Arabia. Saudi J Health Sci [serial online] 2021 [cited 2021 Jul 23];10:38-42. Available from: https://www.saudijhealthsci.org/text.asp?2021/10/1/38/312901

  Introduction Top

Sickle cell disease (SCD) is widespread across Saudi Arabia,[1] with the southwestern region, Aseer, and Jazan, containing the largest sickle cell population in Saudi Arabia.[2] The estimated prevalence of SCD, derived mainly from the national premarital screening program, showed an overall (6.8, 7 for sickle cell anemia [SCA] patients, and 42.7, 135.2 for carriers respectively) per 1000 persons.[2] Malaria, agriculture, and human migration are responsible factors for the current distribution of all hemoglobinopathies; including SCD.[3] High-risk marriages and consanguinity marriage maintained the current prevalence of the disease.[4],[5],[6] According to a recent analysis of the Saudi national premarital screening program, the sickle cell allele showed a stable prevalence compared to decreasing prevalence of thalassemia.[2]

The pathophysiology of SCD is multifactorial and complex. Various environmental stimuli initiate hemoglobin S polymerization, with subsequent vaso-occlusion-induced tissue ischemia. Chronic hemolysis induces endothelial dysfunction with ongoing sterile inflammation leading to multiorgan dysfunction.[7] Due to this multisystem nature of the disease, several international and national guidelines exist to pinpoint the best practices of provisioning suitable care for these patients.[8],[9],[10] Hence, multidisciplinary specialized care service, provided by comprehensive care canters, is suggested. Given the chronic nature of the disease and the associated pattern of complications, patients with hemoglobinopathies represent a real challenge for health care providers.

Currently, very few patients with hemoglobinopathies receive comprehensive care in Saudi Arabia. The 2030 vision includes many initiatives that target new-born screening and the provision of comprehensive care to patients with hemoglobinopathies. Aseer Region, located in southwestern Saudi Arabia, contains the highland region, which is composed of Abha, Khamis Mushait, Sarat Abidah, Namas, Ballahmar, and Ballasmar, and the Tihama region, which is composed of Muhail, Rejal Almaa, Bareq, and Mujardah. There are numerous non-central hospitals, one tertiary hospital along with other governmental, none ministry-of-health hospitals, and private-sector hospitals, which are 34 in total, with more than 3900 inpatient beds. Currently, there is no comprehensive care center for hereditary diseases, and the health care services provided to patients are usually at their nearby peripheral hospitals. Aseer Central Hospital is a tertiary care center located at highland Abha city. Although Abha city has few local sickle cell populations, patients with SCD are also referred to the hospital or come as walk-in to the emergency department (ED). This study aims at reviewing the current pattern of inpatient care provided for patients with SCD admitted to Aseer Central Hospital in ASEER REGION.

  Methodology Top

This was a retrospective chart review study. The study protocol was approved by the hospital institutional review board (REC No. 20190906) and REC of College of Medicine, King Khalid University (HA-06-B-001). Medical records were screened for all admission for patients (more than 12-year-old) with SCD. In order not to miss admissions not labeled for SCD, lab records were also screened for all Hb electrophoreses tests that were done from 2015 to 2019 and results for Hemoglobin S were identified. Chart records were reviewed for all patients with available hemoglobin S results, and data for admission details for patients with SCA were collected in a standardized datasheet and transformed into an excel file. Then, data were entered and analyzed using the Statistical Program for the Social Sciences (SPSS) version 21(IBM Corp., Armonk, N. Y., USA). The standard methods of descriptive statistics were used to describe the data (i.e., frequencies and percentages for categorical variables, mean, and standard deviation for continuous variables). Statistical tests were used for the analysis of the association between categorical variables. Significance was set at P < 0.05.

  Results Top

Excluding sickle cell trait, outpatient only visits, daycare, ED visits, and missing files, 165 admissions were identified for 121 patients. [Table 1] summarizes the patients' characteristics.
Table 1: Bio-demographic data of sickle cell anemia patients (n=1)

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Admissions details

103 (62.4%) were direct admissions from the ED, followed by patients referred from other hospitals 52 (31.5%), and then from the outpatient clinic 10 (6.1%). Most of the admissions were under the care of internal medicine 114 (69.1%), and general surgery 23 (14%). Hematology service was commonly requested for admitted patients (78%). Most patients were admitted to general wards 149 (90%), and only 13 (8%) were admitted to critical care units and 3 (2%) remained in ED for the full duration of the hospitalization.

[Figure 1] summarizes all the admission events and reported complications for this cohort of patients. Vaso occlusive pain crisis (VOC) was the most frequent indication for admission 96 (58%), followed by hepatobiliary surgical conditions 27 (16%), and pulmonary complications 21 (12.7%). Patients came mainly from ASEER highland region 64 (52.5%), followed by the TIHAMA region 34 (27.8%), and the nearby JAZAN region 23 (19.7%) [Figure 2]. [Table 2] summarizes some patients and lab characteristics according to admission types.
Figure 1: Frequency of sickle cell patient's admission and complications

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Figure 2: Admission type per origin of the patients

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Table 2: Patient's personal data and laboratory investigations comparison for selected admission diagnoses

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Blood transfusion

242 units of blood were transfused during 106 (64%) admissions. Most of them were in the order of two units 38 (23%), one unit 29 (17.6%) , 4 units 20 (12.1%) and 3 units 19 (11.5%). Among the patients admitted with VOC, 60 (62.5%) received blood transfusion; 4 transfusion reactions were documented. There were four positives direct antiglobulin test results, and 4 events of incompatible antibody screen related mainly to hyperhaemolysis and delayed hemolytic transfusion reactions.

Medication use

Hydroxyurea (HU) was used in 88 (73%) of patients. Only 30 (25%) patients had a dose of 20 mg/kg or above, and only 6 patients had a dose of 30 mg/kg. Folic acid usage was common (82%) and was usually prescribed at a higher dose of 5 mg/day (79%). Inpatient deep venous thrombosis prophylaxis was also common (85%), mainly with low-molecular-weight heparin (66%). Intravenos paracetamol (81%) and morphine (61%) were the most commonly used analgesic. Protein-pumps inhibitors were commonly used (40%).

The use of antibiotics in inpatient sickle cell patients was frequent (69%); ceftriaxone (40%) was used the most, followed by piperacillin-tazobactam (14.4%), and levofloxacin (11.4%).

Lab results

[Table 3] summarizes the obtained lab results for the reported sickle cell anemia patients. The median Hb level was 8.6 g/dl. Elevated HbA2 (>3.5%) along with microcytosis (MCV < 80 fl) was commonly found in 81 (67%) of patients which is suggestive for the presence of sickle cell-B thalassemia genotype. Whereas the remaining 40 (33%) had a genotype consistent with Hb SS. 20/40 of patients with Hb SS genotypes had microcytosis suggesting the possibility of coexisting alpha thalassemia. 7 patients tested positive for HCV, and 3 tested positive for HBV. No positive HIV case was reported in the studied population.
Table 3: Laboratory investigations for sickle cell anemia patients

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  Discussion Top

This retrospective chart review study in a tertiary care hospital offers a glimpse of the current model of health care provision of patients with SCD. The pattern of disease complications and health service utilization match those reported nationwide where vaso-occlusive episodes remain the most common reason for seeking medical help, followed by hepatobiliary complications, and acute chest syndrome.[11],[12],[13],[14],[15]

Two important aspects of clinical care for patients with SCD were investigated; blood transfusion, and the use of HU, both of which constitute an integral part of the long-term care with proven clinical benefits.

Blood transfusion was common event during sickle cell admissions and most patients admitted for VOC pain crises received blood transfusion, in 60 out of 96 admissions (62.5%). Although our study is retrospective and would not assess the severity of pain episodes, it is expected that those admitted were among the most severe presentations. The mean hemoglobin concentration for those admitted with VOC was 8.5 g/dl (Standard deviation 1.6) which questions the need for blood transfusion for this indication.[16] In general, the use of blood transfusion in the management of SCD-related acute and chronic complications are on the rise,[17],[18] and there is a need to re-investigate the utility of this intervention in order to ensure evidence based utilization.

HU is a very essential drug for the care of SCA patients. Almost all major society guidelines recommend HU, and the label is extended to cover a wide range of indications in both children and adults. Long term follow-up has shown safety, efficacy, decrease morbidity, and improved survival.[19],[20] In our cohort, the underuse is strikingly common, with only quarter of the patients use HU at the recommended starting dose of 20 mg/kg/day, which is consistent with our personal observation. Potential reasons are that those patients were presented with acute complication management and the absence of long-term follow-up in the admitting hospitals for patients coming from nearby regions. Several studies have identified some obstacles to its use including concerns about infertility or carcinogenesis.[21],[22] These concerns and obstacles should be addressed and tackled to ensure the wider use of this important drug.

Regarding the health care provision, the primary care providers were nonspecialists, mainly those of general internists and other nonspecialist surgical departments. Although at our center, SCA patients have access to hematology expertise, this is not the case in the peripheral hospitals, where most of the patients usually present initially. This might explain why about (48%) of patients are coming from TIHAMA and nearby JAZAN regions, and most of those were admitted through the ED. Traveling from an area 80–250 km away from the hospital might imply patient dissatisfaction with the provided nearby care services. Most of the peripheral hospitals in the Aseer region do not have a hematology specialised service due to the shortage in the number of hematologists. Establishing a comprehensive care center is expected to improve accessibility, clinical outcomes, and patients satisfaction.[23],[24] However, the location of a center and the model of care delivery should be wisely addressed to ensure accessibility for patients living in widely apart areas. Telemedicine capabilities have been implemented with success to reach patients living in rural and far reached areas.[25]

Moving ahead, the future seems promising. The ministry of health has multiple initiatives to increase awareness and provide the specialized care to SCD patients. This is coupled with a global recognition of this disease, new breakthrough discoveries, and drugs approval. The new ministry of health initiatives includes implementing newborn screening to early diagnose such disease and start appropriate management of it. The national premarital screening program offers an opportunity for primary prevention; and although high-risk marriage still seems to be high,[5],[26] it can be tackled by proper counseling and education.

  Conclusion Top

The current model of care for patients with SCD in Saudi Arabia is expected to improve in the near future. The two main disease-modifying interventions, blood transfusion and HU therapy, are either misused or underused. For both interventions, there is a need for constant examinations to ensure the maximum benefit of these important tools of management.

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Conflicts of interest

There are no conflicts of interest.

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  [Figure 1], [Figure 2]

  [Table 1], [Table 2], [Table 3]


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