Disease management refers to the management of care needs and maintaining quality of life.
The progressive nature of ALS requires periodic reevaluation of care needs. The wishes of the individual with ALS may change over time, sometimes only slightly, or possibly significantly.
Comorbidity: the presence of one or more additional medical conditions alongside a primary condition.
Progressive: a condition that tends to worsen over time, as in ALS.
Prognosis: a forecast or prediction about the likely course and outcome of a disease or condition.
Prognostication: the process of determining a prognosis.
Disease progression in ALS is measured using a numerical scale known as ALSFRS-R. A lower ALSFRS-R score implies a worse health condition and further progression of the disease.
The ALSFRS-R is readily available online. Individuals with ALS can complete the questionnaire regularly, such as every month, to track their progression.
Accurately predicting an individual's prognosis with ALS, including specific health details and timing of eventual death, is challenging due to the significant variability in the rate of disease progression.
An individual with ALS might experience a period of rapid progression that later slows down, leading them to believe their disability will increase quickly and that their death is imminent. Conversely, someone with initially slow-progressing ALS may suddenly experience a rapid acceleration of symptoms that they may find themselves unprepared for.
While forming accurate long-term prognoses can be challenging, making short- and medium-term predictions is possible and reasonable. For instance, if an individual who could easily walk upstairs a month ago is now struggling, it is reasonable to predict that they might not be able to climb stairs at all within the next few months.
Guiding principles are general concepts for the individual's family and care team to follow. Each individual with ALS will place differing amounts of value on prolonging their survival, enjoying the present time, and their willingness to access and use medical and other resources.
Some aspects of a person's lifestyle and activity choices might involve risks that could be seen as excessive, yet they may be important for supporting the individual's mental health.
The individual should consider and communicate the following key aspects:
Their level of interest in accessing drug treatments, including approved and experimental options.
Their risk tolerance when it comes to partaking in activities that could cause serious injury.
Their willingness to pursue treatments for medical conditions associated with ALS, such as breathing and nutrition needs.
Their willingness to pursue treatments for medical conditions not associated with ALS (comorbidities).
ALS has no cure, however, there are many types of treatments that directly affect survival. Such treatments include:
An individual with ALS may choose to pursue or forgo any number of treatments, depending upon their survival wishes. The individual should make clear what level of intervention they are seeking so that their care team knows when to administer or withhold treatments.
Individuals with comorbidities that impact their overall survival may need to make decisions about whether to pursue or forgo treatments for them.
Tracheostomy and tube feeding can theoretically prevent death being directly caused by ALS. Instead, the individual would eventually die of some other cause, such as from infection, a comorbidity, or an accident.
An individual that is relying on life-saving support such as ventilation or tube feeding may have some control over their survival by voluntarily removing these supports.
ALSWiki.org does not provide information on assisted suicide. Consult the applicable government's website for information.
An individual with ALS should clearly communicate their treatment and survival wishes to their care team and family. Their wishes are subject to change at any time; it is within their right to do so. Open lines of communication between all people involved should be maintained. Quality of life is highest when all parties understand and work together to meet the needs and wishes of the individual with ALS.