Sickle Beta Zero Thalassemia, Sickle B+ Thalassemia, Sickle Cell Disease, Sickle Cell Hemoglobin C
Conditions
Brief summary
This project addresses three important research questions. First, adolescents and young adults (AYA) with sickle cell disease (SCD) and their parents/caregivers will be engaged to inform the (1) domains of health-related quality of life (HRQOL) most important to them, (2) frequency at which they are willing to complete them, and (3) other procedures related to the use, uptake and effect of the HU-Go app as a tool to improve hydroxyurea (HU) adherence. Second, this study seeks to utilize novel modern mobile technology using a multi-functional personalized platform to improve adherence to HU and measure HRQOL in youth with SCD, using NIH-endorsed PROMIS® measures, based on a conceptual model with predefined behavioral targets and mediators. Third, we plan to assess HRQOL changes and identify modifiable behavioral strategies that could serve as surrogates or predictors for HU adherence. This real-time feedback might empower self-directed changes in behavior that could improve adherence to HU.
Detailed description
Aim 1: Identify the needs, desires, concerns, and expectations of AYA patients with SCD and their parents/caregivers that will guide implementation of a mHealth tool, HU-Go, designed to improve adherence to HU. We will conduct semi-structured interviews with patients and their parents/caregivers. Transcripts will be independently coded and thematic analysis will be conducted. Hypothesis 1: AYA patients with SCD and their parents/caregivers will identify specific features, content, and usability requirements of HU-Go. Aim 2: Assess the longitudinal relationship of HU adherence to HRQoL domains, including fatigue and depression. HU adherence will be measured using electronic pill bottles, self-report measures, laboratory markers, and medication possession ratio (MPR). HRQOL will be assessed using PROMIS® measures. Hypothesis 2: Low HU adherence is associated with impairment of HRQoL domains. Aim 3: Conduct a single-arm, pilot study to determine the effect size associated with improvement in HU adherence after using HU-Go for 12 weeks. Adherence will be measured using self-report measures, laboratory markers and MPR. Hypothesis 3: HU-Go will improve HU adherence by at least 20%.
Interventions
OTHERHU-Go app
A novel multifunctional mobile app (HU-Go) to improve adherence to hydroxyurea in patients with sickle cell disease
Sponsors
Ann & Robert H Lurie Children's Hospital of Chicago
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
SUPPORTIVE_CARE
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
12 Years to 25 Years
Healthy volunteers
No
Inclusion criteria
* At least 12 years old at the time of study enrollment * Diagnosis of sickle cell disease (hemoglobin SS or SC or S/B 0 thalassemia) confirmed by hemoglobin electrophoresis * On hydroxyurea * Own or have access to a smartphone * Be able to speak and read English
Exclusion criteria
* Chronic monthly transfusion support * Any hemoglobinopathy other than sickle cell disease
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Achieving feasibility based on the number of participants completed all study procedures within 6 months of study enrollment | 3 months of study enrollment | Feasibility is defined as having 80% or more of study participants complete the study within 6 months of enrollment |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Adherence to Hydroxyurea using Modified Morisky Adherence Scale 8-items | 3 months of study enrollment | Numerical value on a scale 0-8 (higher score indicating higher adherence to hydroxyurea) |
| Adherence to Hydroxyurea using Visual Analogue Scale | 3 months of study enrollment | Numerical value on a scale 0-100% (higher score indicating higher adherence to hydroxyurea) |
| Patient satisfaction with the smartphone app intervention (HU-Go) | 3 months of study enrollment | Customized patient satisfaction questionnaire, numerical value on a scale 0-10 (higher score indicating higher satisfaction with the app) |
| HRQOL outcomes | 3 months | Patient reported outcomes measurement information system (PROMIS) measures, numerical value on a scale of 0-100 points, normal average for the general population is 50 with standard deviation of 10 points. Each HRQOL outcome domain score will be reported separately (higher T scores indicating worse pain, fatigue, depression and anxiety, and lower scores indicating worse physical functioning and peer relationships). |
Countries
United States
Outcome results
None listed