Table 1.

Previous studies describing psychological profile of patients/caregivers with CGD

Author, country, year (reference)	Total number of CGD patients/families [total patients with PID]	Median age of participants with CGD (years) [range]	Psychosocial scales/outcome measures used	Determination of impact of COVID-19 pandemic	Remarks
Cole et al., UK, 2013 [18]	73/47 §	§§	PedsQL SDQ	Not applicable	Patients and caregivers/parents of children with CGD have lower quality of life and poor emotional health; however, those who undergo HSCT have comparable emotional health to healthy controls
Battersby et al., UK, 2019 [19]	75/62* [75]	43 [3–77]	SF-36 V2 HADS	Not applicable	All XL-CGD carriers had reduced quality of life Among XL-CGD carriers, 26/61 and 5/61 reported moderate-to-severe anxiety and depression respectively Higher anxiety scores were directly proportional to depression scores, low self-esteem, presence of arthritis or bowel disease, and increased fatigue
Pulvirenti et al., Italy, 2019 [13]	47/47 [47]	7.3 ± 4.4** 27.6 ± 8.0***	PedsQL SDQ SF-12	Not applicable	Children with CGD reported more problems in social/school areas, peer relationship, and conduct/emotional problems as compared to controls Adults with CGD also reported higher difficulties both in mental and physical domains as compared to controls No significant difference was noted between patients who underwent HSCT versus those who did not
Deshpande et al., USA, 2020 [20]	NA [565] #	NA	PROMIS	Yes	Of the 565 patients with PIDs, 543 (96%) had primary antibody defects Nearly 63% (359/565) patients with PIDs had issues with accessing healthcare during COVID-19 pandemic Mean PROMIS global physical (39.6) and mental health scores (43.5) were reduced as compared to the national population Patients who had healthcare access problems more commonly reported a moderate-to-severe negative impact on their physical and mental health vs those without access issues
Sowers et al., USA, 2021 [11]	1/1 [511]	NA	Questionnaire (Qualtrics survey platform)	Yes	< 2% of patients with PID reported positivity for COVID-19 infection About 30.9% (n = 158) were “extremely concerned” with the possibility that their ward or family member would contract COVID-19 infection and 16.8% (n = 86) reported disruption of daily activity due to COVID-19 Nearly half (n = 292) reported that they were “mostly isolated” during the pandemic About 56.6% (n = 289) utilized telemedicine to contact primary physician managing their ward with PID
Manusama et al., Netherlands, 2022 [21]	1/1 [176]	NA	4-DSQ	No	Patients with PIDs had more numbers of “moderate” and “high” scores in all 4 domains, i.e., distress (33.9% vs 16.3%), depression (18.6% vs 5.7%), anxiety (22.4% vs 8%), and somatization (36.2% vs 11.2%) as compared to controls
Meelad et al., Malaysia, 2022 [22]	1/1 [10]	19	Semi-structured interview	No	Parents/caregivers had concerns on 5 thematic aspects: (1) living with fear and anxiety; (2) struggles of PID healthcare system; (3) knowledge about disease; (4) social constraint; and (5) coping
Akdag ˘ et al., Turkey, 2022 [9]	0/0 [65]	NA	GAD-7 PTSD checklist civilian version	Yes	Parents/caregivers of patients with PID reported higher rates of moderate-to-severe anxiety in comparison to parents with healthy children (67.7% vs 32.1%) PTSD checklist civilian scores were also higher in parents/caregivers of patients with PID (p < 0.05)

CGD, chronic granulomatous disease; COVID-19, coronavirus disease-2019; PedsQL, Pediatric Quality of Life Inventory; SDQ, Strengths and Difficulties questionnaire; HSCT, hematopoietic stem cell transplantation; SF-36 V2, Medical Outcomes Study Short Form 36 version 2; HADS, Hospital Anxiety and Depression Scale; SF-12, 12-item Short Form health survey; PROMIS, Patient-reported Outcomes Measurement Information System; PID, primary immunodeficiency disorder; 4-DSQ, 4-Dimensional Symptom Questionnaire; GAD-7, generalized anxiety disorder 7-item; PTSD, post-traumatic stress disorder; NA, not available

^§Forty-seven and 42 parents completed PedsQL and SDQ questionnaires, respectively, and 35 children completed self-report PedsQL questionnaires

^§§In the group where parents completed PedsQL, median age was 9 years ((range 3–15) for non-HSCT) and 10 years ((range 4–14 years) for post-HSCT). In the group where children completed PedsQL, median age was 10 years (range 5–15) for both non-HSCT and post-HSCT. In the group where parents completed SDQ, median age was 9 years for non-HSCT and 10 years for post-HSCT

^*X-linked carriers of CGD: 75

^**Mean age of pediatric patients

^***Mean age of adult patients

^#Total no. of patients with primary immunodeficiencies: 565