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IntroductionPrimary immunodeficiency diseases (PIDs) are chronic diseases that affect the various aspects of a patient’s life. However, the impact of living with PIDs is poorly described.ObjectiveThis study aimed to explore the living experience challenges among the Malaysian caregivers of the patients with PID who underwent a follow-up in the Universiti Sains Malaysia or those registered members of the Malaysian PIDs Society.MethodologyThe study was conducted from March 1 to May 30, 2021. The parents of children with PIDs were invited to participate for a semi-structured in-depth interview at the PID clinics in the USM. The estimated time of each interview was 30 min. The semi-structured interview was performed via a telephone call because of COVID-19 pandemic restrictions. The audio recording of each interview was transcribed and translated from Malay to English. Subsequently, a thematic analysis utilizing the ATLAS.ti software was performed.ResultsThe thematic analysis revealed five main themes, which are living with fear and anxiety with four sub-themes (sickness, psychological issues, fear of infections and hereditary issues), PID healthcare support struggles with four sub-themes (PID health system, treatment, diagnosis and financial issues), knowledge with two sub-themes (educational issues and disease understanding), social constraint with two sub-themes (relationships and social isolations) and coping with three sub-themes (acceptance, child health improvement and emotional hygiene).ConclusionLiving with fear and anxiety is a major theme impacting the living experiences of Malaysian patients with PIDs. Improvements in healthcare delivery and disease education are needed to ensure optimal quality of life.

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.