Engagement in CHildhood-Onset Systemic Lupus

Feasibility of the Valera health application will determined by the percentage of participants who utilize the Valera platform. Participants with repeated (i.e., ≥ 2) log-ins to both the care management and educational platforms will be considered users. A successful target percentage of ≥ 80% of enrolled participants logging into the care plan on the application and utilizing the messenger system at least twice during the 6-month pilot will be considered highly feasible.

Feasibility of the Intervention Measure (FIM) will be assessed by participant rating of application feasibility via administration of a 4-item questionnaire at the end of the pilot study. Responses to each of the 4 questions will be evaluated on a 5-point scale as follows: (1 = "Completely disagree", 2 = "Disagree", 3 = "Neither agree nor disagree", 4 = "Agree", 5 = "Completely agree"). A higher aggregate score denotes a more favorable participant rating of feasibility of the application.

Acceptability of the Intervention Measure (AIM) will be assessed by participant rating of application acceptability via administration of a 4-item questionnaire at the end of the pilot study. Responses to each of the 4 questions will be evaluated on a 5-point scale Response (1 = "Completely disagree", 2 = "Disagree", 3 = "Neither agree nor disagree", 4 = "Agree", 5 = "Completely agree"). A higher aggregate score denotes a more favorable participant rating of the application. For purposes of this study a group mean score of ≥4 (representing "Agree" or "Completely agree") across the 4 items in ≥80% or participants will be an indicator of Acceptability.

Engagement in Care will be defined by the number of participants who have completed at least 1 clinical cSLE visit during the 6-month pilot window.

Change from baseline in patient reported lupus disease activity will be assessed using the SLE Activity Questionnaire (SLAQ). The SLAQ is an assessment tool which consists of 24 questions related to the severity and symptoms of lupus over the prior 30 days rated on a Likert-like scale ranging from 0 ("No problem") to 3 ("Severe") as well as a single Patient Global Assessment question about presence of lupus activity disease activity over the prior 30 days scored on a scale of 0 ("No activity") to 10 ("Most activity"). For purposes of this study change from baseline is being assessed such that a positive value signifies increased lupus disease activity from baseline and a negative value signifies decreased lupus disease activity from baseline.

Change from baseline in patient reported lupus disease activity will be assessed using the SLE Activity Questionnaire (SLAQ). The SLAQ is an assessment tool which consists of 24 questions related to the severity and symptoms of lupus over the prior 30 days rated on a Likert-like scale ranging from 0 ("No problem") to 3 ("Severe") as well as a single Patient Global Assessment question about presence of lupus activity disease activity over the prior 30 days scored on a scale of 0 ("No activity") to 10 ("Most activity"). For purposes of this study change from baseline is being assessed such that a positive value signifies increased lupus disease activity from baseline and a negative value signifies decreased lupus disease activity from baseline.

Change from baseline in clinically assessed lupus disease activity as defined by the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2K). SLEDAI-2K is a scoring system based on the (binary) presence or absence of 24 descriptors in nine organ systems assessed over the preceding 30 days. Each of the descriptors has a weighted score and the total score of SLEDAI-2K is the sum of all 24 descriptor scores (range 0-105). For purposes of this study change from baseline is being assessed such that a positive value represents increased SLE disease activity relative to baseline based on the SLEDAI 2K and a negative value represents decreased SLE disease activity relative to baseline based on the SLEDAI 2K.

Change from baseline in clinically assessed lupus disease activity as defined by the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2K). SLEDAI-2K is a scoring system based on the (binary) presence or absence of 24 descriptors in nine organ systems assessed over the preceding 30 days. Each of the descriptors has a weighted score and the total score of SLEDAI-2K is the sum of all 24 descriptor scores (range 0-105). For purposes of this study change from baseline is being assessed such that a positive value represents increased SLE disease activity relative to baseline based on the SLEDAI 2K and a negative value represents decreased SLE disease activity relative to baseline based on the SLEDAI 2K.

Change from baseline in patient reported depression symptoms severity as defined by Beck Depression Inventory-II (BDI-II). This questionnaire consists of a 21-item self-reporting inventory answered by the participant based on a 4-point scale of increased severity of depression symptoms over the prior 2-week period. The BDI score is based on a 4-point scale, with 0 indicating no impairment and 3 indicating severe impairment for a possible overall scoring range of 0-63. For purposes of this study change from baseline is being assessed such that a positive value represents increased depression symptom severity relative to baseline based on BDI-II and a negative value represents decreased depression symptom severity relative to baseline based on the BDI-II.

Change from baseline in patient reported depression symptoms severity as defined by Beck Depression Inventory-II (BDI-II). This questionnaire consists of a 21-item self-reporting inventory answered by the participant based on a 4-point scale of increased severity of depression symptoms over the prior 2-week period. The BDI score is based on a 4-point scale, with 0 indicating no impairment and 3 indicating severe impairment for a possible overall scoring range of 0-63. For purposes of this study change from baseline is being assessed such that a positive value represents increased depression symptom severity relative to baseline based on BDI-II and a negative value represents decreased depression symptom severity relative to baseline based on the BDI-II.

Change from baseline in patient reported depression symptoms severity will be assessed using the PHQ-9. The PHQ-9 is a 9-item self-reporting inventory answered by the participant related to the severity of their depression symptoms over the prior 2-week period. As a severity measure, responses to the PHQ-9 questions are scored on a 4-point scale ranging from 0 ("Not at all") to 3 ("Nearly every day") and a composite score is summed from the individual items. For purposes of this study change from baseline is being assessed such that a positive value represents increased depression symptom severity relative to baseline based on the PHQ-9 and a negative value represents decreased depression symptom severity relative to baseline based on the PHQ-9.

Change from baseline in patient reported depression symptoms severity will be assessed using the PHQ-9. The PHQ-9 is a 9-item self-reporting inventory answered by the participant related to the severity of their depression symptoms over the prior 2-week period. As a severity measure, responses to the PHQ-9 questions are scored on a 4-point scale ranging from 0 ("Not at all") to 3 ("Nearly every day") and a composite score is summed from the individual items. For purposes of this study change from baseline is being assessed such that a positive value represents increased depression symptom severity relative to baseline based on the PHQ-9 and a negative value represents decreased depression symptom severity relative to baseline based on the PHQ-9.

Change from baseline in patient reported perceived stress severity will be assessed using a Perceived Stress Scale 10 item questionnaire. Participants are asked to provide responses to each question based on their feelings and thoughts related to stressors over the prior month. Responses to the 10 items are scored on a 5-point scale ranging from 0 ("Never") to 4 ("Very Often") and a composite score is summed from the individual items. For purposes of this study change from baseline is being assessed such that a positive value signifies an increased level of perceived stress relative to baseline and a negative value signifies a decreased level of perceived stress from baseline.

Change from baseline in patient reported perceived stress severity will be assessed using a Perceived Stress Scale 10 item questionnaire. Participants are asked to provide responses to each question based on their feelings and thoughts related to stressors over the prior month. Responses to the 10 items are scored on a 5-point scale ranging from 0 ("Never") to 4 ("Very Often") and a composite score is summed from the individual items. For purposes of this study change from baseline is being assessed such that a positive value signifies an increased level of perceived stress relative to baseline and a negative value signifies a decreased level of perceived stress from baseline.

Change from baseline in self-efficacy will be assessed using the Patient Reported Outcome Measurement Information System (PROMIS) Self-efficacy treatment and medication questionnaire. This 26-item questionnaire measures patient confidence in taking medications and handling therapy in the context of potential challenges. Responses to each item are scored on a 5-point Likert-like scale ranging from 1 ("I am not at all confident") to 5 ("I am very confident"). Raw itemized scores are aggregated and transformed to a T-score using a linear transformation. The T-score is centered at 50 with a standard deviation of 10 such that 50 is considered average. Higher scores (e.g., 60) indicate greater patient confidence in management of medications and therapy relative to a general chronic illness population and lower scores (e.g., 40) indicate lower patient confidence in management of medications and therapy relative to a general chronic illness population. T-scores will be summarized by arm.

Change from baseline in stigma will be assessed using the Patient Reported Outcome Measurement Information System Neurological Quality of Life (PROMIS Neuro-QOL) short form questionnaire. The PROMIS Neuro-QOL is an 8-item survey that measures patient-reported perceived disease-related stigma. Responses to each item are scored on a 5-point Likert-like scale ranging from 1 ("Never") to 5 ("Always"). Raw itemized scores are aggregated and transformed to a T-score using a linear transformation. The T-score is centered at 50 with a standard deviation of 10 such that 50 is considered average. Higher scores (e.g., 60) indicate greater perceived stigma relative to a general chronic illness population and lower scores (e.g., 40) indicate lower perceived stigma relative to a general chronic illness population. T-scores will be summarized by study arm using basic descriptive statistics.