Burden of vaso-occlusive crisis, its management and impact on quality of life of Indian sickle cell disease patients

Abstract

Sickle cell disease (SCD) with vaso-occlusive pain crisis (VOC) significantly impacts patient well-being and often results in extensive healthcare resource utilization. This study assessed the VOC burden, its management and its impact on patients' quality of life (QoL). A cross-sectional observational study was conducted between November 2021 and June 2022, including 1000 SCD patients from high-prevalence states in India. Data on demographics, clinical characteristics, VOC severity, management and QoL were collected. The study revealed that 33.5% of patients reported at least one VOC episode during the study period. In the year prior to their enrolment, 836 (83.60%) patients reported at least one VOC episode, with an equal proportion of 407/487 (83.6%) adults and 429/513 (83.6%) paediatric patients, reducing their QoL across all domains compared to patients without VOC. Of these, 469/1000 patients (46.9%) experienced ≥3 VOC episodes. Additionally, 764/1000 (76.40%) patients managed their VOCs at healthcare facilities, with 501/1000 (50.1%) requiring inpatient admissions. Further, 71.80% of patients received Hydroxyurea (HU) therapy. The study depicts the severity of the Arab-Indian haplotype in Indian SCD patients visiting healthcare settings based on high VOC burden. This highlights the urgent need for better management strategies and resource allocation for these patients.

Keywords: healthcare utilization; quality of life; sickle cell disease; vaso‐occlusive crisis.

FIGURE 1

Patient recruitment strategy. (A) Shows one out of three patients with VOC. During the study period, different SCD patients 1 (A), 2 (B), and 3 (C) up to 1000 visited the hospital for various reasons like VOC, routine follow‐up and blood transfusion. All the patients visiting the hospital who provided the informed consent were screened for eligibility criteria and assigned a unique identification number. All variables pertaining to the study were captured on the first (index) visit. (B) Shows one patient with VOC at the index visit* who experienced another VOC at the third hospital visit. During the study period between the index visit of the 1st patient and the index visit of the 1000th patient, the same patient (e.g. patient A in the figure) might have visited the hospital multiple times for various reasons (i.e. VOC, follow‐up, blood transfusion). For such patients, on the index visit, a unique identification number was assigned post‐screening for eligibility, and all the variables pertaining to the study were captured. During the patient's subsequent visits to the hospital, limited data pertaining to the reason for a hospital visit, such as VOC, its severity, category (complicated/uncomplicated) and organ involved, were documented during the study period. (C) Flow diagram for the disposition of 1000 patients with SCD for the study period between the 1st patient's index visit: Nov 24, 2021, and the 1000th patient index visit: June 28, 2022.*Index Visit: The patient's visit to the study site where the patient provides informed consent and all the data about the study are captured for the first time.

FIGURE 2

Regional VOC distributions. (A) Regional distribution of VOC in India, (B) Regional distribution of VOC episodes across India based on HU therapy. N represents the total number of patients from the region. n, represents the number of patients who experienced at least one VOC in the year prior to enrolment. Data represented as n (%).

FIGURE 3

Analysis of the quality of life of SCD patients reporting VOC episodes versus those reporting no VOC in the year prior to enrolment. (A) Mean health utility scores from EQ‐5D‐5L questionnaire (N = 1000). (B) Mean ASCQ‐Me scores from 487 adult SCD patients. (C) Heat map of the proportion of SCD paediatric patients' responses to the paediatric‐specific SCD questionnaire. n represents the number of patients in each group. Data represented as Mean ± Standard deviation of health utility scores. (Independent sample t‐test **p < 0.01, ***p < 0.001).