Quality of life related to clinical features in patients with Rett syndrome and their parents: a systematic review

Patients with a neurodevelopmental disorder such as Rett syndrome (RS), as well as their families, have complex needs that affect their quality of life (QoL). Therefore, both families and patients with RS must be provided with multidisciplinary health care that can identify the clinical features that most affect their QoL and mental health risks. The main objective of this paper is to provide a comprehensive overview of the QoL subdimensions of families affected by RS, including both the parents and children. We conducted a systematic review, following PRISMA criteria, of the data in the PubMed, PsycINFO, Cuiden, and LILACS databases. The results indicated that when considering the family as a whole, RS equally affects the physical and psychological QoL dimensions; the next most affected was the social dimension. According to parents’ reports, seizures are one of the main factors that decreases their QoL. Thus, from a clinical point of view, controlling seizure activity of children with RS is the main way of improving the QoL of their parents. Interventions in patients affected by RS should be based on the improvement of visual contact and concentration, reducing somnolence, and increasing mobility. The subdimensions of QoL that were most affected in parents of girls with RS were those related to mental health and feelings of well-being.