This information is for health professionals and service providers.
The effects of MND – initial symptoms, rate and pattern of progression, and survival time after diagnosis – vary significantly from person to person.
With no effective treatments as yet available to stop the relentless progression of MND, effective and timely symptom management from diagnosis through to bereavement is vital to quality and length of life.
This section of the MND Connect website provides health professionals and service providers with excerpts from and links to latest research papers, evidence based reviews, conference presentations and practice, or expert consensus, guidelines related to the myriad symptoms and support needs of people living with ALS/MND. The aim is to support a coordinated multi/inter disciplinary team approach to symptom management. The papers below confirm the benefits of this approach in improving survival and quality of life.
The papers featured are not exhaustive and the MND Australia team will endeavour to update information in this section on a regular basis. Please contact the MND Australia team if you would like to share a relevant paper, guideline or presentation.
Currently, the mainstay of care for patients with ALS is timely intervention to manage symptoms, including use of nasogastric feeding, prevention of aspiration (control of salivary secretions and use of cough-assist devices), and provision of ventilatory support (usually with bilevel positive airway pressure) (Brown and Al-Chalabi 2017).
On average, death results within 2–3 years from symptom onset. Characterized by heterogeneous patterns of deterioration, presenting symptoms range from falls, limb weakness, communication, and swallowing difficulties to changes in mood, cognition, and behavior. Up to 50% of people with ALS develop some degree of frontotemporal impairment. Patients may also be at risk of psychological problems such as depression and anxiety, linked to their disease experiences. No cure exists for ALS, and only a small number of treatments (eg, riluzole, non-invasive ventilation) delay death. Death usually results from respiratory failure. Care delivered and coordinated by multidisciplinary clinics (MDCs) specializing in ALS has been shown to extend survival and improve the quality of life for patients (Hogden et al 2017).
Patients with ALS should be provided opportunities for multidisciplinary care since the spectrum of available treatments can markedly impact both quality of life and survival. ALS should no longer be considered “untreatable” based on the broad range of interventions that can be prescribed for symptom management (Jackson et al 2015).
The diagnosis of ALS has profound implications for the patient and his or her family. In a recent analysis, too few patients received evidence-based treatment that can ease the disease burden. Although incurable at this time, advances in contemporary care options available to the patient with ALS have been shown to prolong life and also to improve the quality of that life.
These AAN ALS quality measures have been developed to address gaps in patient care and to overcome the underutilization of evidence-based therapeutics for patients with ALS. The measures are concerned with supportive care, management of patient safety, and planning for complex care needs, using a patient-centered approach to proactive decision making.
The AAN ALS quality measures encourage multidisciplinary care plans, treatments for respiratory and nutritional dysfunction, use of the single disease-modifying agent available, and plans for a smooth transition to palliative care. Multidisciplinary clinics are available to some patients, and, where available, referral is encouraged. The addition of practical and meaningful quality measures for the care of patients with ALS may raise the standard of care and lead to the desired outcome, an increased life expectancy with an enhanced quality of life (Miller and others 2013).