adolescents and young adults, advance directives, goals of care, neuromuscular disease, palliative care



  1. Battista, Vanessa DNP, MBA, MS, RN, CPNP-PC, CHPPN
  2. Baker, Deborah J. DNP, ACNP, NEA-BC
  3. Trimarchi, Tara DNP, RN, CRNP
  4. Sabri, Bushra PhD, MSW
  5. Wright, Rebecca PhD, BSc (Hons), RN
  6. D'Aoust, Rita F. PhD


The use of advance directives is an important component in helping individuals living with chronic and/or life-threatening illnesses establish goals of care and make decisions regarding care at the end of life. Advance care planning may help achieve enhanced health outcomes, yet it is not routinely offered to adolescents/young adults living with neuromuscular disease. An integrative review of the literature was conducted to examine the evidence related to the use of advance directives with adolescents/young adults living with neuromuscular disease and to identify reasons why they are not being used and how this can be improved. Three-hundred-seven studies were retrieved from PubMed, CINAHL, and EMBASE. Five studies met the final inclusion search criteria and were included in the analysis. Four themes emerged from the literature: conversations about advance directives with adolescents/young adults with neuromuscular disease are not being conducted, only a small number of patients have documented advance directives, patients want to have conversations about goals of care and want to have them sooner, and there is a lack of evidence in this area. These findings may influence neuromuscular clinicians' practice surrounding the use of advance directives and increase their knowledge regarding the need for discussions regarding goals of care.