BACKGROUND: Perioperative nursing care for patients with neuromuscular disorders, especially spinal muscular atrophy (SMA), remains a challenge. There is an obvious lack of guidelines. METHODS: We retrospectively reviewed the medical charts of patients with type II or III SMA who underwent spinal surgery from 2018 to 2022. Nursing assessments included muscle strength, pulmonary function, the Barthel Index, the Braden Scale, Nutrition Risk Screening 2002, and the Hamilton Anxiety Scale. Preoperative and postoperative anxiety levels were compared using a paired-samples t-test. RESULTS: All 24 included patients had severe scoliosis, kyphosis, or kyphoscoliosis, with a mean Cobb angle of 102 degrees. Upon admission, all patients (24/24) presented with muscle weakness, were classified as having total or severe dependency, and were at risk of developing pressure sores; 58.3% (14/24) of the patients had severe pulmonary function impairment, and 50.0% (12/24) were at nutritional risk, with the score unable to be assessed in 8.3% (2/24) of the patients. All patients underwent posterior spinal fusion surgery with bone grafting. Only one patient experienced a major postoperative complication, pneumonia, which was effectively managed. Anxiety level decreased significantly (P < 0.01) at discharge compared to that on admission. Complementing regular nursing care, an SMA-specific perioperative nursing care protocol was implemented: (1) Respiratory care protocol: A. Confirmation of SMA type; B. Comprehensive evaluation of symptoms, signs, and pulmonary function test results; C. Development and implementation of a personalized plan including: Plan 1. Training on respiratory function including diaphragmatic breathing exercise, coughing exercise, inhaling exercise, and exhaling exercise; Plan 2. Use of cough assist device, and/or Plan 3. Use of non-invasive ventilator. (2) Postoperative three-step all-involved training protocol of postural adaptation from nurse-led to caregiver-led and inducing patient self-advocacy: A. Preparation for the training; B. Postural adaptation training; C. Postural switch from lying to sitting. CONCLUSIONS: We implemented an SMA-specific perioperative nursing care protocol, including a respiratory care protocol and a postoperative three-step all-involved training protocol of postural adaptation, complementing standard nursing care. Our approach yielded positive patient outcomes, while we acknowledge the limitation that our protocol is pending comparative evaluations due to the rarity of the disease. The protocol was initially designed for patients with SMA but may also be suitable for other patients with profound muscle weakness.