Minerva Pediatrica 2020 Nov 13

DOI: 10.23736/S0026-4946.20.05844-2

Copyright © 2020 EDIZIONI MINERVA MEDICA

lingua: Inglese

The characteristics of self-care in children with spinal muscular atrophy: an integrative review

Federico RUTA ¹ ✉, Barbara BASSOLA ¹, Maura LUSIGNANI ²

¹ University of Rome Tor Vergata, Rome, Italy; ² University of Milan, Milan, Italy

BACKGROUND: Spinal muscular atrophy (SMA) is one of the most common genetic causes of death in children. Recently, European Commission (EU) has approved a new gene therapy based on onasemnogene abeparvovec (Zolgensma) for the treatment of patients with SMA. It is essential that children suffering from SMA also apply self-care methods to maintain their health, monitor their weight and food intake, and use appropriate remedies. Indeed, self-care is a crucial element in the health care system because it is able to improve survival and prevent hospitalizations. The purpose of this review is to systematically explore the characteristics of self- care in children with spinal muscular atrophy and the relationship with their parents and the collaboration of healthcare professionals.
METHODS: An integrative review of the literature has been conducted. The electronic databases CINAHL, Embase, PubMed, and SCOPUS were searched.
RESULTS: Thirteen articles met the inclusion criteria and were reviewed using Whittemore and Knalf’s integrative review methodology. The analysis of the 13 articles selected for the review show that previous literature has focused on six prevalent themes: problem solving and care behavior, decision making, optimizing living with an illness, high quality, childfocused homecare, healthcare professionals, and monitoring behaviors.
CONCLUSIONS: This paper highlights how self-management behaviors depend on four basic aspects: the person (individual, cognitive, and social perceptions), the patient’s family (level of knowledge of the pathology, involvement in the management and quality of relationship with the patient), the community (relationships with external social contexts, such as school and other organizations), and the healthcare system (availability of resources and the degree of evolution of healthcare).

KEY WORDS: Chronic disease; Decision-making process; Spinal muscular atrophy; Self-care; Children