A qualitative study of the impact of plexiform neurofibromas on need fulfilment in adults with neurofibromatosis type 1

Objective: To explore the impact of plexiform neurofibromas on the lives of adults with neurofibromatosis type 1. Background: Neurofibromatosis type 1 is a complex neurogenetic syndrome that affects many aspects of health and functioning. A common manifestation of neurofibromatosis type 1 is plexiform neurofibromas, non-cancerous tumours that can cause disfigurement, pain and neurologic disability. Patient-reported outcome measures used in this condition have addressed symptoms and functional ability but not how the condition affects patients’ lives, particularly, their ability to meet their human needs. Methods: Unstructured qualitative interviews were conducted with adults with neurofibromatosis type 1–associated plexiform neurofibromas in the United Kingdom and United States. Interviewees were encouraged to describe how plexiform neurofibromas affected their ability to meet their needs. Interviews were audio-recorded and transcribed verbatim. The UK and US transcripts were combined and theoretical thematic analysis was conducted. Results: In all, 42 interviews (United Kingdom = 20, United States = 22) were conducted. Transcripts revealed 696 statements on the impact of plexiform neurofibromas on need fulfilment. Five major themes emerged: appearance, relationships, independence, role fulfilment and pleasure. Conclusion: Neurofibromatosis type 1–associated plexiform neurofibromas have a major effect on individuals’ ability to meet their needs. An understanding of need fulfilment will complement information generated from traditional patient-reported outcome measures, particularly in a multi-faceted syndrome such as neurofibromatosis type 1

Towards climate resilient and environmentally sustainable health care facilities

The aim of building climate resilient and environmentally sustainable health care facilities is: (a) to enhance their capacity to protect and improve the health of their target communities in an unstable and changing climate; and (b) to empower them to optimize the use of resources and minimize the release of pollutants and waste into the environment. Such health care facilities contribute to high quality of care and accessibility of services and, by helping reduce facility costs, also ensure better affordability. They are an important component of universal health coverage. Action is needed in at least four areas which are fundamental requirements for providing safe and quality care: having adequate numbers of skilled human resources, with decent working conditions, empowered and informed to respond to these environmental challenges; sustainable and safe management of water, sanitation and health care waste; sustainable energy services; and appropriate infrastructure and technologies, including all the operations that allow for the efficient functioning of a health care facility. Importantly, this work contributes to promoting actions to ensure that health care facilities are constantly and increasingly strengthened and continue to be efficient and responsive to improve health and contribute to reducing inequities and vulnerability within their local settings. To this end, we propose a framework to respond to these challenges

The development of the PlexiQoL: A patient‐reported outcome measure for adults with neurofibromatosis type 1‐associated plexiform neurofibromas

ABSTRACT Background To develop and validate a patient‐reported outcome (PRO) measure of quality of life (QoL), specific to patients with Neurofibromatosis Type 1 (NF1)‐associated plexiform neurofibromas (pNFs), suitable for use in clinical efficacy trials. The study was conducted in parallel in the UK and US. Methods Qualitative interviews were conducted with patients to generate questionnaire content. Face and content validity of the draft scale was assessed by cognitive debriefing interviews (CDIs). A postal validation survey was conducted to identify the final version of the questionnaire (the PlexiQoL), establish its unidimensionality, and assess its psychometric properties. Results Thematic analysis was performed on 42 interview transcripts. Thirty‐one CDIs revealed that patients found the draft scale to be comprehensible, relevant, and easy to complete. The postal validation survey was completed by 273 patients. Rasch analysis identified an 18‐item unidimensional scale that showed excellent internal consistency, reproducibility, and sensitivity to differences in patient‐perceived pNF severity, general health, and the use of pain medication. Conclusions The PlexiQoL is the first disease‐specific PRO assessing the ability of adults with NF‐1 associated pNFs to meet their basic human needs. Clinical trials are planned to assess the sensitivity to change of the PlexiQoL in people undergoing treatment for pNFs