Health-Related Quality of Life of Patients and Families with Primary Immunodeficiency in Malaysia: A Cross-Sectional Study

Abstract

Purpose: Primary immunodeficiency disease (PID) affects various aspects of a patient’s life. However, the health-related quality of life (HRQOL) of PID patients in Malaysia patients is poorly described. This study aimed to determine the quality of life of Malaysian PID patients and parents. Method: This cross-sectional study was performed from August 2020 to November 2020. Patients with PID and their families were invited to answer the PedsQL Malay version (4.0) questionnaire, the tool used to assess the HRQOL. A total of 41 families and 33 patients with PID answered the questionnaire. A comparison was performed with the previously published value of healthy Malaysian children. Result: Parent of respondents recorded a lower mean of total score than the parents of normal healthy children (67.26±16.73 vs. 79.51±11.90, p-value = 0.001, respectively). PID patients reported lower mean total score to normal healthy children (73.68 ± 16.38 vs. 79.51 ± 11.90, p-value = 0.04), including psychosocial domain (71.67±16.82 vs. 77.58±12.63, p-value = 0.05), and school functioning, (63.94±20.87 vs. 80.00 ± 14.40, p-value = 0.007). No significant difference of reported HRQOL when comparing between subgroup of PID on immunoglobulin replacement therapy and those without immunoglobulin replacement (56.96±23.58 vs. 65.83±23.82, p-value 0.28). Socioeconomic status was found to be predictive of the lower total score of PedsQL in both parent and children reports. Conclusion: Parents and children with PID, especially those from middle socioeconomic status, have lower HRQOL and school function impairment than healthy children.

Impact of Primary Immunodeficiency Diseases on the Life Experiences of Patients in Malaysia From the Caregivers’ Perspective: A Qualitative Study

“…The list of the caregivers of PID patients who had participated in a previous health-related quality-of-life (QOL) study was included ( 6 ). Patients with low QOL scores (mean < 67.26; defined as those parents or caregivers with a total score that is lower than the mean of the total score of the PID cohort studied as a comparison for cut-off points for low QOL scores) were identified and invited to participate in the present qualitative study ( 6 ).…”

Paving the way in implementation of SCID newborn screening in developing nations: feasibility study and strategies to move forward in Malaysia

“…It was reported that children who receive immunoglobulin replacement therapy (IGRT) recorded low school functioning compared to those without IGRT. This is primarily due to the need for frequent hospital visits and other health-related issues, which adversely affect the overall quality of life for these children (59). It is proven that utilizing NBS/ENBS for SCID screening leads to significant benefits.…”