Importance of therapeutic patient education in ichthyosis: results of a prospective single reference center study

Abstract

BackgroundIchthyoses are a heterogeneous group of rare genodermatoses. Patients and their families face difficulties related to daily care and management that may be aggravated by social isolation.ObjectivesTo evaluate the impact of therapeutic educational programs in improving the knowledge of ichthyosis patients, and their relatives, about their disease.Patients and methodsWe organized a two sessions-program of “know-how” dedicated to the overall management of ichthyoses. These sessions were conducted based on a tool specifically designed for the study, which addressed our various areas of expertise through a collective game. The participants (patients and their parents and siblings) were divided into groups, and the questions were tailored according to the participants’ age. The program was conceived as a knowledge reinforcement program that took place during a weekend of education and rest, organized away from healthcare structures. Our aim was to facilitate the program in a neutral place to encourage respite care and to ensure the availability of a multidisciplinary healthcare team.ResultsAfter the reinforcement session, children aged from 6 to 12 years and their families acquired the targeted know-how and social skills.ConclusionBenefits of TPE in the management of ichthyoses are the following: (1) the trust between patients their families and the caregivers was strengthened; (2) the context of the program encouraged self-expression, answered questions and provided mutual aid; and (3) the more self-sufficient families could better manage emergencies.

Assessing the quality of life in the families of patients with epidermolysis bullosa: The mothers as main caregivers

“…In addition, the study demonstrated that the psychological and social aspects of the life of patients with autosomal recessive congenital ichthyoses and their families were affected by this disease ( Abeni et al, 2021 ). Moreover, Dufresne et al (2013) found that therapeutic educational programs in a neutral place (out of routine therapeutic and health care centers) for patients with ichthyoses improved the patients’ and their families’ knowledge and social skills, which could be considered in future studies on Iranian patients with EB. Furthermore, in a study by Sjedianfard et al (2021) on FQoL in patients with pemphigus vulgaris, FQoL was shown to be poorer in families with male patients, older patients, and shorter duration of disease, as well as in lower-educated caregivers.…”

Ichthyosis (concept, pathohistology, clinical picture, treatment)

“…Ни дети, ни их родители зачастую не понимают, как можно предотвратить эти двигательные и сенсорные осложнения. Цель терапевтического образования больных детей состоит в том, чтобы помочь им лучше понять свою болезнь, необходимость выполнения ежедневного ухода за кожей и других процедур для их выживания и комфорта [44,45]. Кожные симптомы оказывают значительное влияние на качество жизни пациентов, но мало что известно о бремени (психологическом, социальном, экономическом, физическом) этого заболевания на семьи пациентов.…”

Management of congenital ichthyoses: European guidelines of care, part one

“…Educational interventions (‘ichthyosis schools’) may be very useful to improve treatment adherence and lessen fears and misconceptions (LoE 3, GoR D) . Nevertheless, formal and structured multidisciplinary educational programmes have been established in a minority of European countries, and there are very few data evaluating their impact . Patients must be informed about the national patient support groups that exist in many European countries and allow support from other families and sharing of individual experiences (LoE 4, GoR D).…”