Long-Term Ventilation in Neuromuscular Patients: Review of Concerns, Beliefs, and Ethical Dilemmas

Abstract

Background: Noninvasive mechanical ventilation (NIV) is an effective treatment in patients with neuromuscular diseases (NMD) to improve symptoms, quality of life, and survival. Summary: NIV should be used early in the course of respiratory muscle involvement in NMD patients and its requirements may increase over time. Therefore, training on technical equipment at home and advice on problem solving are warranted. Remote monitoring of ventilator parameters using built-in ventilator software is recommended. Telemedicine may be helpful in reducing hospital admissions. Anticipatory planning and palliative care should be carried out to lessen the burden of care, to maintain or withdraw from NIV, and to guarantee the most respectful management in the last days of NMD patients’ life. Key Message: Long-term NIV is effective but challenging in NMD patients. Efforts should be made by health care providers in arranging a planned transition to home and end-of-life discussions for ventilator-assisted individuals and their families.

Posicionamiento de la Sociedad Española de Neumología y Cirugía Torácica en el uso de la telemedicina en los trastornos respiratorios del sueño y ventilación mecánica

“…Arch Bronconeumol. 2020. https://doi.org/10.1016/j.arbres.2020.05.032 También, como en la CPAP, se puede incorporar la transmisión de señales para una monitorización remota de los pacientes mediante plataformas web que recogen datos diarios suministrados telemáticamente por los equipos 74 . De esta manera se pueden efectuar, si es preciso, intervenciones, como llamadas telefónicas.…”

Transition from Childhood to Adulthood in Patients with Duchenne Muscular Dystrophy

“…Positive airway pressure (PAP) is preferred in the treatment of OSA, and biphasic or bilevel positive airway pressure (BIPAP) is preferred when respiratory failure occurs [ 40 , 41 ]. It is a long-term therapy, and in the era of telemedicine, can be safely conducted in the patient’s home [ 42 ].…”

The adult multidisciplinary respiratory neuromuscular clinic

“…In younger people in particular, there appears to be a reluctance from both caregivers and clinicians to initiate conversations about end-of-life care with the patient [105,106]. The evidence base to support the inclusion of palliative care is sparse [107,108], restricting access to resources. This is partly because, unlike in oncology or COPD, "end-stage" NMD has not been categorically defined [109], leading to clinical challenges about when to introduce palliative care.…”