Sickle cell disease (SCD) is an invalidating monogenic red cell disorder, characterized by acute pain during vaso-occlusive crisis (VOC). Most of the VOCs is manage at home, requiring support from the nurse-medical staff of the comprehensive sickle cell centers. In addition, sickle cell related acute pain management at home might be unsatisfactory and difficult to be tracked. Here, we developed and validated a medical app named SCD MED ALERT for home management of acute pain in patients with SCD. SCD MED ALERT is a multilingual and standalone app for iOS and Android, pain episodes are easily inserted, and records are held locally on the device. Following checks and therapy reminders are sent via the device calendar. SCD MED ALERT was proposed to young adult patients with SCD (n=47), referring to comprehensive sickle cell centers of Società Italiana Talassemie ed Emoglobinopatie (SITE), with > 2 VOCs in the last 6-8 months, referring to comprehensive centers for hemoglobinopathies. SCD patients were 60% female, with mean (SD) age 32.2 (10.3) years and the following genotypes: 51.1% SS, 25.5% Sβ, 14.9% SC, 8.5% AS. 17/47 (36%) patients were under chronic pain treatment, such as non-steroidal anti-inflammatory drugs (12, 25.5%), paracetamol 4.0 (8.5%), |opiates 3.0 (6.4%) and |other forms of analgesia 3.0 (6.4%). No major difference was observed both in gender and age distribution for either chronic pain treatment (treated females 11/28, 39%, males 6/19, 32%, p=0.8; treated 31.4±10.7 years, not-treated 32.7 ±10.3, p=0.69), for crizalizumab plus hydroxyurea therapy (treated females 5/28, 18%, males 4/19, 21%, p=1; treated 27.8±9.5 years, not-treated 33.3 ±10.3, p=0.15), for hydroxyurea alone (treated females 24/28, 86%, males 13/19, 68%, p=0.29; treated 32.5±10.2 years, not-treated 31.4 ±11.3, p=0.79) or for chronic erythroexchange (treated females 11/28, 39%, males 8/19, 42%, p=1; treated 33.9±11.4 years, not-treated 31.1 ±9.5, p=0.39).

Patient Activation Measure-13 (PAM-13) questionnaire was administrated at baseline and at 2 months, whereas mHealth App Usability Questionnaire (MAUQ) was evaluated at 2 months of use.

In SCD patients using SCD MED ALERT, the mean PAM score was 3.02 (0.32) at the baseline and 3.23 (0.33) at 2 months of use (p<0.001). The total PAM-score increased from 39.3 (4.2) to 42.0 (4.3) (p<0.001). When we considered SCD patients under medical treatments, the PAM-score at the baseline showed a positive association with patients on crizalizumab plus hydroxyurea (HU) (p=0.015), suggesting a higher sense of self-efficacy in SCD patients under combination treatment than on HU alone: PAM-score 44.4 (3.4) vs 38.7 (3.9), p=0.0034. We noted a positive association of the difference of PAM-score for patients on chronic erythroexchange (p=0.036). MAUQ questionnaire showed a positive consensus of SCD patients on the use of application with a mean score of 6.0 (0.6). Overall, 31/44 (70%) answering patients used the app to report VOC crises. There was no effect of gender or of chronic pain treatment on either PAM-score at baseline and after SCD MED ALERT use. SCD patients contributed with the following main suggestion to improve SCD MED ALERT app: “medication reminders and trackers for chronic treatments” (n=23), “information material on the disease” (n=10) possibility of communicating with other patients (n=10) and “possibility of communicating with healthcare personnel (n=9).

Our study shows that SCD MED ALERT app increases patient sense of self-efficacy and pro-activity in home pain management during acute VOCs. SCD MED ALERT app allowing the early treatment of sickle cell related of pain, might contribute to the empowerment of patients with SCD.

Disclosures

Pinto:Vertex: Membership on an entity's Board of Directors or advisory committees; BMS: Membership on an entity's Board of Directors or advisory committees. Origa:BMS: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau.

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2024
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