**Background:** Neuromuscular disorders (NMDs) are a heterogeneous group of conditions causing muscle dysfunction, often affecting respiratory function. While conditions like amyotrophic lateral sclerosis (ALS) progress rapidly over 2–4 years, others like Duchenne muscular dystrophy (DMD) progress over three to four decades. Optimizing respiratory function can improve quality of life and survival, but progression continues despite interventions. Palliative care, defined as active holistic care for life-limiting illness, is often underutilized in NMDs, with only 14% of those needing palliative care receiving it globally. This review focuses on applying palliative care principles to NMD patients with respiratory involvement, using six themes from Janisch et al. (2020): management of complex symptoms, crisis support, relief of caregiver strain, coordination of care, advance care planning, and end-of-life care.
**Methods:** A systematic search was conducted in PubMed/MEDLINE, CINAHL, and the Cochrane Database of Systematic Reviews using three concepts: NMDs (including specific conditions), palliative/end-of-life care, and respiratory failure/ventilator support. Primary studies of any design in English were included, along with review articles and opinion pieces due to limited primary research. Supplementary searching used reference lists and emerging themes. Integrative narrative synthesis was used to develop recommendations based on the six themes.
**Key Results:** Initial searches identified 323 citations; after title/abstract review, 155 remained, of which 41 were original research. Many papers were reviews, guidelines, or opinion pieces. Original research covered service models, population studies, advance care planning, symptom management, and patient/carer perspectives. Key findings include:
- **Symptom management:** Multidisciplinary proactive assessment is central. Common but often unaddressed symptoms include pain, fatigue, constipation, and low mood. For breathlessness, low-dose morphine can relieve symptoms without compromising respiratory function. Secretion management includes physiotherapy, mechanical insufflation-exsufflation, mucolytics (e.g., carbocysteine), and anticholinergics (e.g., hyoscine, glycopyrronium). Pain management involves multidisciplinary approaches, with short courses of low-dose opioids or neuropathic agents (e.g., gabapentin, duloxetine) when needed. Fatigue management is multimodal, addressing sleep, diet, exercise, depression, and pain. Depression and anxiety are prevalent and should be treated proactively.
- **Crisis support:** Patients with NMDs are at risk of rapid deterioration, often from acute respiratory illness. Parallel planning—hoping for the best while planning for the worst—is recommended. Emergency hospital admissions remain consistent across all ages in DMD, even near death.
- **Caregiver strain:** Carer burden is widely acknowledged. In one study, 30% of caregivers of ventilated ALS patients rated their quality of life lower than the patient's. Support for siblings and practical respite (e.g., children's hospices) is important, but age-appropriate respite for young adults is often lacking.
- **Coordination of care:** Good communication and coordinated care across community, social care, and medical specialties are valued. Transition from pediatric to adult services is crucial, especially as conditions progress.
- **Advance care planning (ACP):** ACP should begin before interventions are considered, with regular reviews. Condition-specific tools (e.g., traffic light systems) can identify when palliative care is needed. Discussing sudden death and resuscitation preferences is important but challenging.
- **End-of-life care:** Essential elements include recognizing dying, sensitive communication, involving the patient and family, and an individual care plan covering symptom control, food/drink, and psychosocial/spiritual support. Specific considerations for NMDs include feeding/fluids (may need to stop in last days), symptom management (often poorer than in cancer), communication support, and ventilator management. Ventilator withdrawal requires careful planning, with opioids and midazolam titrated to manage breathlessness. Guidelines from the Association of Palliative Medicine and pediatric palliative care emphasize planning, communication, and psychosocial support.
**Clinical Implications:** Palliative care should be introduced early in NMDs based on need, not prognosis. All clinicians should develop skills in generalist palliative care, and specialist palliative care should be embedded in the neuromuscular multidisciplinary team to facilitate education and timely referral. Research gaps remain, particularly as new life-prolonging treatments emerge.