Nursing

Gene

Best practice

Context (archaeology)

Cognitive psychology

Delphi method

Health care

Medical home

Biology

Statistics

Economics

Biochemistry

Rett syndrome

Paleontology

Psychology

Mathematics

Management

Primary care

Checklist

Family medicine

Medicine

Chemistry

Economic growth

BMJ Paediatrics Open

Background Rett syndrome (RTT) is a severe neurodevelopmental disorder with complex medical comorbidities extending beyond the nervous system requiring the attention of health professionals. There is no peer-reviewed, consensus-based therapeutic guidance to care in RTT. The objective was to provide consensus on guidance of best practice for addressing these concerns. Methods Informed by the literature and using a modified Delphi approach, a consensus process was used to develop guidance for care in RTT by health professionals. Results Typical RTT presents early in childhood in a clinically recognisable fashion. Multisystem comorbidities evolve throughout the lifespan requiring coordination of care between primary care and often multiple subspecialty providers. To assist health professionals and families in seeking best practice, a checklist and detailed references for guidance were developed by consensus. Conclusions The overall multisystem issues of RTT require primary care providers and other health professionals to manage complex medical comorbidities within the context of the whole individual and family. Given the median life expectancy well into the sixth decade, guidance is provided to health professionals to achieve current best possible outcomes for these special-needs individuals.

Consensus guidelines on managing Rett syndrome across the lifespan