The 2018 SMA Standards of Care recommendations were published in Neuromuscular Disorders in February 2018 and March 2018 and are available online through Open Access for families and healthcare providers. These documents are updates of the Standard of Care document issued in 2007.
The 2018 recommendations emphasize that “a multidisciplinary approach is the key element in the management of SMA patients.” For the best outcomes in SMA patients, healthcare providers representing a varierty of specialties should work together across specialties and with families.
The first FDA-approved therapy for SMA, Spinraza, has made care management of SMA more important than ever. Access to treatments must be combined with improved care to optimize the impact of treatments, extend lifespan and enhance quality of life for those affected by SMA.
Dr. Eugenio Mercuri, Italy, Dr. Richard Finkel, US, and Dr. Thomas Sejersen, Sweden, led this effort and convened international experts to review SMA care evidence. Cure SMA provided funding support for this project and Mary Schroth, Chief Medical Officer for Cure SMA, served on the workgroup.
Part 1 describes the methods used to achieve the recommendations and an update on how to best diagnose SMA and how to best manage musculoskeletal, orthopedic, nutrition, swallowing and gastrointestinal problems and bone health. Part 2 provides updated best management recommendations for breathing, illness care, at home and in the hospital, and sedation, ethical considerations and palliative care, and new topics including involvement of other organ systems and the role of medications.
Diagnosis and management of spinal muscular atrophy: Part 1: Recommendations for diagnosis, rehabilitation, orthopedic and nutritional care. Neuromuscular Disorders 28 (2018) 103–115.
Diagnosis and management of spinal muscular atrophy: Part 2: Pulmonary and acute care; medications, supplements and immunizations; other organ systems; and ethics. Neuromuscular Disorders 28 (2018) 197-207.
Our thanks to the members of the SMA Care Workgroup for their dedication and expertise on this project.