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Updated: Jun 3, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Knowledge, Attitude, and Practice of Patients Suffering From Sickle Cell Disease in an Endemic Zone
Garima Nirmal1, Dinesh Pendharkar2, Neha Gupta2
1Departments of Pediatric Oncology, Haemato-Oncology.
Background And Objective:
Sickle cell disease (SCD) is a genetically inherited disorder that is associated with morbidity and mortality.
Methods:
This cross-sectional study was conducted on patients diagnosed with SCD to evaluate the knowledge, attitude, and practice of patients/guardians using a pretested questionnaire.
Results And Discussion:
Of the 111 participants, 56 (50.4%) were male. Forty-five participants reported SCD as a hereditary disease, and only 31 (37%) subjects reported that SCD could be prevented. Fourteen (70%) married patients reported that their children had been screened. Ninety-three (84%) children reported fatigue in the past 1 year. Seventy-four of them reported no absence from work/school in the past 1 year. Fifty-seven participants (52%) experienced 1 to 4 episodes of pain in the past 1 year, 62 and the majority (55.86%) of them visited a nearby doctor for the pain episode. Ninety-four participants were taking regular hydroxyurea as medication, and 72% had not missed any dose in the past 1 month, while 14.4% missed 1 to 2 doses in the past 1 month. Only 19 participants (17%) were aware that transplantation was a curative option. The majority (78%) reported undergoing blood and other investigations irregularly in the preceding year.
Conclusions:
The knowledge and attitude of patients/guardians regarding the nature of the disease, preventive measures, curative options for transplantation, and screening of family members are low. At the same time, most people are educated. In contrast to the world data, adherence to hydroxyurea is extremely good. Patient and family education and counseling are the needs of the hour.
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