Self-Reported Medication Adherence Measured with Morisky Scales in Rare Disease Patients: A Systematic Review and Meta-Analysis.

BACKGROUND: The visibility of Rare Diseases is a new challenge for society. These diseases are numerous, heterogeneous in nature and distribution, characterized by a high mortality rate but low prevalence, and usually presenting a severe evolution. Adherence to medication studies in rare diseases are uncommon, due to treatment scarcity. OBJECTIVES: The main purpose of this study is to do a meta-analysis, evaluating the level of adherence to medication in the most prevalent rare diseases. METHODS: This work is a systematic review, and meta-analysis was registered in the International Prospective Register of Systematic Reviews (PROSPERO) (Registration number: CRD42022372843) and conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Adherence to treatment in this systematic review and meta-analysis was collected from all studies included, based on the crude numerators and denominators reported, using either the Morisky Medication Adherence Scale 4 or -8. RESULTS: A total of 54 records were identified through database searches, or after screening relevant manuscripts' references. Finally, 18 studies were included in this systematic review and meta-analysis. A total of 1559 participants (54.18% women) aged less than 84 years old were included. Twelve studies used the MMAS-8. In 8 of them, they established the level of adherence to treatment in three categories (low, medium, and high), with the mean prevalence in each of them being 41.4%, 30.4%, and 28.2%, respectively. CONCLUSIONS: The results observed in adherence to treatment in patients with rare diseases show great variability, due to the different aspects involved in the greater or lesser applicability of the medication.

Enfermedades raras desde la farmacia comunitaria / Rare diseases from the community pharmacy

Introducción: la visibilidad de las Enfermedades Raras a nivel multidisciplinar y su humanización, son uno de los retos planteados en la Agenda 2030 de Naciones Unidas. El farmacéutico comunitario necesita renovarse y promover habilidades, como técnicas de atención farmacéutica individualizada, imprescindibles para el apoyo de estos pacientes. El objetivo de esta investigación es identificar las necesidades socio-sanitarias de las familias perte-necientes a asociaciones de enfermedades raras que acude a la farmacia comunitaria en España.Método: Estudio observacional y transversal me-diante cuestionario digital enviado a usuarios de la Asociación de Enfermedades Raras D ́genes, a través de la Asociación y de los Colegios Oficiales de Farmacéuticos, n=253.Resultados: las enfermedades raras se distribuyen por todo el territorio con una alta dispersión. La mayoría de pacientes o familiares suelen visitar la farmacia comunitaria más de dos veces al mes. Estas familias suelen confiar en su farmacia desde hace más de tres años y no encuentran dificultades para obtener su tratamiento. El perfil de los pacien-tes que acuden a la farmacia es muy heterogéneo, pero la mayoría de familiares afirman conocer y centrarse únicamente en sus necesidades sociosa-nitarias. En relación a sus niveles de satisfacción, el recibimiento, la discreción en el trato, el tiempo de atención y la despedida son los factores mejor valorados.Conclusiones: El profesional farmacéutico debe poseer, habilidades científicas y técnicas, necesita promover valores como empatía, habilidades socia-les o capacidad de escucha, para poder prestar una atención farmacéutica personalizada a familias con enfermedades raras y conseguir humanizar la farmacia comunitaria del siglo XXI.(AU)

Introduction: the visibility of Rare Diseases at a multidisciplinary level and their humanization are one of the challenges set out in the 2030 Agenda of the United Nations. Community pharmacists need to renew themselves and promote skills, such as in-dividualized pharmaceutical care techniques, which are essential for the support of these patients. The aim of this research is to identify the socio-health needs of families belonging to rare disease asso-ciations who go to the community pharmacy in Spain.Method: Observational and transversal study by a digital questionnaire to users of the Association of Rare Diseases D'genes, through the Associa-tion and the Official Associations of Pharmacists, n=253.Results: Rare diseases are distributed throughout the territory with a high dispersion. Most patients or relatives usually visit the community pharmacy more than twice a month. These families usually trust their pharmacy for more than three years and do not encounter difficulties in obtaining their treatment. The profile of the patients who visit the pharmacy is very heterogeneous, but the majority of family members claim to know and focus only on their social and health needs. In relation to their levels of satisfaction, the reception, discretion in treatment, time of attention and farewell are the best valued factors.Conclusions: The professional pharmacist must possess, scientific and technical skills, needs to promote values such as empathy, social skills or listening skills, to be able to provide personalized pharmaceutical care to families with rare diseases and manage to humanize the community pharma-cy of the 21st century.(AU)