Impact of a digital manual for guidance on malignant hyperthermia: patient education

Author(s)

Rodrigues, Gislene ; de Andrade, Pamela Vieira ; dos Santos, Joilson Moura ; Gomes do Amaral, Jose Luiz ; Almeida da Silva, Helga Cristina

• Document type: Journal article
• Source: ORPHANET JOURNAL OF RARE DISEASES; v. 17, n. 1, p. 9-pg., 2022-07-15.

Abstract

Background Malignant hyperthermia (MH) is a rare, hereditary disease with a hypermetabolic response to volatile anesthetics/succinylcholine. Susceptible patients face difficulties due to a lack of knowledge about MH. As informational materials could increase knowledge and adherence to prevention/therapy, digital information about rare diseases validated for patients is needed. Our objective was to evaluate the following: (1) the impact of digital manuals on the knowledge/quality of life of MH patients and (2) access to MH services. Materials and methods Fifty MH-susceptible patients filled out a virtual questionnaire twice (demographic/economic/clinical data, MH knowledge and impact on daily life, and SF-36 quality of life). Test groups 1 (n = 17) and 2 (n = 16) were evaluated 30 and 180 days after receiving a digital manual, and the control group (n = 17; without manual) was evaluated after 180 days. We collected the MH service data about the number of contacts. Results Twenty-four (48%) patients reported problems in personal/professional life, sports, clinical/surgical/dental treatments, and military service, in addition to concerns about emergency care and complaints of sequelae. The percentage of correct answers in the second MH knowledge questionnaire increased for test group 2 (62% vs. 74.1%; unpaired t test, p < 0.01), was significantly greater in test groups 1 (68.1%) and 2 (74.1%) than in the control group (56.5%; Kruskal-Wallis, p < 0.05), and correlated with more time studying the manual and reports of MH-related problems (multiple regression, p < 0.05). Conclusions The digital manual improved patients' MH knowledge. Online contacts with the MH service increased, allowing greater information dissemination. As informational materials could increase knowledge/adherence to prevention/therapy, digital information about MH validated for patients should be implemented. (AU)

• FAPESP's process: 21/06180-7 - Malignant hyperthermia: Brazilian multidisciplinary study
• Grantee: Helga Cristina Almeida da Silva
• Support Opportunities: Regular Research Grants