What is the Survivability of ALS in Australia?

The **survivability of ALS (amyotrophic lateral sclerosis) in Australia** reflects a complex interplay of factors including disease subtype, patient age, behavioral symptoms, and emerging treatments. ALS is a progressive neurodegenerative disease that affects motor neurons, leading to muscle weakness, respiratory failure, and ultimately death. Survival rates vary widely, but recent advances in treatment and understanding of the disease have begun to improve outcomes for some patients.

Typically, the **median survival time after ALS diagnosis globally ranges from 2 to 5 years**, but this can differ based on individual factors. In Australia, survival patterns appear consistent with international data, but ongoing research and clinical trials are contributing to a more nuanced understanding and potential improvements in longevity.

One of the most promising developments in Australia is the use of a repurposed veterinary drug called **NUZ-001**, which has shown to significantly extend survival and slow disease progression in ALS patients. In a long-term study, patients treated with NUZ-001 experienced about a 1.5-year longer survival compared to historical controls, along with a 31% slower decline in motor function and a 43% slower decline in lung function. This treatment was well tolerated and maintained quality of life and cognitive function over nearly three years of use. Such findings offer hope for improved survivability in the Australian ALS community as further trials are conducted.

Survival in ALS is also influenced by behavioral and cognitive symptoms. Research indicates that patients exhibiting **apathy** as a behavioral symptom tend to have a more rapid functional decline and shorter survival times. This highlights the importance of comprehensive clinical assessment beyond just motor symptoms to better predict prognosis.

Age at disease onset is another critical factor. Younger patients tend to survive longer, a trend observed in Asian populations and likely applicable in Australia as well. The median age of onset in ALS is typically in the 50s to 60s, and younger onset correlates with slower disease progression and better survival.

Biomarkers such as **urinary p75ECD** and **neurofilament light chain** in blood are being studied to track disease progression and predict survival, although baseline levels of some biomarkers like urinary p75ECD do not directly correlate with survival time. These biomarkers may help in tailoring treatments and monitoring patient response over time.

Other treatments available in Australia, including **Edaravone (marketed as Radicava ORS)**, have demonstrated survival benefits in clinical studies, extending survival by slowing functional decline. These treatments, combined with multidisciplinary care approaches involving respiratory support, nutritional management, and physical therapy, contribute to improved quality of life and potentially longer survival.

In summary, while ALS remains a devastating disease with limited curative options, the **survivability of ALS patients in Australia is gradually improving** due to advances in pharmacological treatments, better understanding of disease subtypes and behavioral impacts, and enhanced supportive care. Survival times vary widely, but new therapies like NUZ-001 and Edaravone offer hope for extending life and maintaining function longer than previously possible.