Health supervision and anticipatory guidance in adult myotonic dystrophy type 1.

The complexity and variability of disease manifestations in myotonic dystrophy (DM1) pose a challenge for the clinical management of patients. The follow-up of DM1 patients has been described as fragmented, inadequate or even deficient for many patients. Through a systematic review of the medical and social literature and a validation process with a DM1 expert panel, we summarized systemic and social concerns clinically relevant to DM1 and revisited recommendations for treatment. This article summarizes common manifestations of the central nervous system, visual, respiratory, cardiac, gastro-intestinal, genito-urinary, muscular and metabolic impairments. In addition, we emphasized the social features of DM1 such as low education attainment, low employment, poor familial and social environment and poor social participation. While cardiac, respiratory and swallowing problems affect life expectancy, it is often excessive daytime sleepiness, fatigue, gastro-intestinal and cognitive behavioural manifestations that are the most disabling features of the disorder. A more holistic approach in the management of DM1 and a purposeful integrated organization of care involving all members of the patients' environment including family, clinicians, decision-makers and community organizations are needed to move out of the spiral of disease and handicap and move toward optimal citizenship and quality of life.

[Nursing care of elderly persons: reactions to the use of a security blanket to prevent falling out of bed]. / Soins infirmiers aux personnes âgées: réactions à l'utilisation de la couverture de sécurité dans la prévention des chutes de lit.

This article presents the results of the first part of a survey aiming at assessing the chances for adoption and use of the safety blanket, a new device preventing the falls from the beds. In this part, the resarchers wanted to know how the caretakers reacted to the use of this material. Thirty four people with five beneficiaries among them, nine family members, fifteen contributors and five managers, interacting in the context of a care unit for elderly people of a hospital centre were interviewed. The data of the interviews were analysed according to a six step procedure: listening to the interviews and reading the descriptions; deriving the significant statements, analysing and reformulating the meaning of the statements; regrouping the signification units under more global themes; gathering the analysis results and describing exhaustively the studied phenomenon; validating the exhaustive description. As a whole, the reactions recorded were positive and indicate that the safety blanket has big chances to be adopted by the healthcare units.