The novel SCD MED ALERT mHealth app: empowering patients with sickle cell disease

Villaboni S;Pinto VM;Gianesin B;Casale M;Perrotta S;Mazzi F;Ceolan J;Pirrone R;Cavellini P;Gigante A;Calvillo M;Origa R;Forni GL;De Franceschi L

2025-01-01

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Abstract

Aim: Sickle cell disease (SCD) is a debilitating monogenic disorder of red blood cells, characterized by acute pain episodes during vaso-occlusive crises (VOCs). Here, we developed and validated a medical app, SCD-MED-ALERT, for home management of acute pain in patients with SCD. Methods: The SCD-MED-ALERT app is a multilingual and standalone application for iOS and Android. It was proposed to young adult patients with SCD (n = 47) attending comprehensive sickle cell centers within the Società Italiana Talassemie ed Emoglobinopatie (SITE) network. Patient-Activation-Measure-13 (PAM-13) questionnaire and mHealth App Usability Questionnaire (MAUQ) were used to evaluate patient activation levels in self-management and the app’s usability, respectively. Results: Among SCD patients using SCD-MED-ALERT, the total PAM score significantly increased from 40 ± 4 (mean ± SD) to 42 ± 4 (P < 0.001). Multivariate analysis revealed that the baseline PAM score was positively associated with patients on crizanlizumab plus hydroxyurea therapy (P = 0.015), suggesting a higher sense of self-efficacy among those receiving combination treatment compared to those on hydroxyurea alone. A positive association was also observed between changes in PAM scores and patients undergoing chronic erythroexchange (P = 0.036). The MAUQ results showed a favorable consensus regarding the app’s usability, with a mean score of 5.9 ± 0.3, reflecting high usability. Overall, 31/44 (70%) responding patients used the app to report pain crises. Neither gender nor chronic pain treatment influenced the PAM scores at baseline or after app use. Conclusion: Our study shows that the SCD-MED-ALERT app enhances patients’ sense of self-efficacy and proactivity in managing pain at home during acute pain crises. We propose that this app might contribute to empowering SCD patients and assist physicians in identifying patients who may benefit from more intensive treatment(s).