¶ Introduction
ALS is a complex disease that affects the body and overall health in many ways. Various treatments and interventions may become available as the disease progresses. An individual with ALS can choose to pursue or decline these options based on their personal life goals and values.
Additionally, activities that are typically low-risk can pose significant dangers for someone with ALS. It's important to carefully evaluate and consider these risks based on the health situation at that time.
¶ Key Terms
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Comorbidity: the presence of one or more additional medical conditions alongside a primary condition.
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Prognosis: a forecast or prediction about the likely course and outcome of a disease or condition.
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Prognostication: the process of determining a prognosis.
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Progressive: a condition that tends to worsen over time, as in ALS.
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Risk tolerance: an individual's ability and willingness to endure uncertainty or negative outcomes when taking actions.
¶ Disease Progression
¶ Measurement
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.
¶ Prognostication
¶ Early stages of ALS
Accurately predicting an individual's prognosis with ALS in the early stages, including specific health details and timing of eventual death, is challenging due to the significant variability in the rate of disease progression.
A sustained period of rapid disease progression may create the impression that severe disability or even death is imminent. Conversely, relatively slow disease progression may make it seem that preparations for increased disability and care needs do not need to take place yet. These assumptions would be invalidated by changes in the rate of disease progression.
While forming accurate long-term predictions is unrealistic, making short- and medium-term predictions is possible and reasonable. For instance, if an individual who could easily climb up stairs 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 couple months.
¶ Later stages of ALS
In the later stages of ALS, prognostication becomes more accurate. An individual experiencing severe respiratory difficulties, an inability to consume enough food to maintain a healthy body weight, or frequently developing infections is more likely to be approaching the end of life.
¶ Treatment Decisions
ALS has no cure, however, there are many types of treatments that directly affect survival. Such treatments include:
- Disease-modifying drugs to attempt to slow down the effects of ALS
- Non-invasive ventilation to assist with early breathing difficulties
- Invasive ventilation (tracheostomy) to assist with advanced breathing difficulties
- Tube feeding to maintain adequate nutrition when independent eating is no longer possible
- Treatments for comorbidities such as infection, injury, and so forth
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.
¶ Risk Tolerance
An individual with ALS has the same right to take personal risk as any other living person. The risk profile for a given activity differs for someone with ALS when compared to someone without the disease, but the right to make those decisions remains with the individual.
Woman with ALS parasailing.
Certain activities, such as driving an automobile on a public road, are not an individual decision; most governments have regulations on levels of physical ability required to maintain a valid driver's license.
A person with ALS should take the time to consider how different activities might impact their health. Situations such as being left alone, experiencing a fall or accident, or choking may not affect a healthy individual at all, but could lead to serious health complications for someone with ALS.
¶ End of Life
¶ Involuntary loss of life
Respiratory failure, pneumonia, or other types of infections are the most common causes of death in ALS. These could occur at any time, but become more likely as the disease progresses. The individual would not have control of their passing in such a case.
A serious comorbidity, such as cancer or heart failure may also cause involuntary loss of life.
¶ Voluntary removal of supports
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.
¶ Assisted suicide
✖ ALSWiki.org does not provide information on assisted suicide. Consult the applicable government's website for information.
¶ Examples
Examples are provided using fictional people.
¶ Meaghan
Meaghan is 45 and has two young children. She is determined to see them both graduate high school. Recently, she found out that she was approved to participate in a drug trial for a promising new treatment for ALS. She is eager to get started and accepts the risks. If she experiences significant respiratory difficulties in the future, she will get a tracheostomy to help prolong her survival and hopefully see her kids graduate.
¶ Adam
Adam is 85 years old and has rapid-onset bulbar ALS. He does not have any children and his wife passed away many years ago. He lives in the countryside with minimal access to health services. Adam has a good memories in the area, and does not want to move to the city. He's happy with how his life has gone and will not be pursuing treatments or interventions for ALS; instead he will live out his last days at home.
¶ Holly
Holly is 27 years old and recently married. She has always been a hard worker, and has come a long way in her career in such a short time. Shortly after diagnosis, she began taking an approved disease-modifying drug and is able to manage the side effects without much difficulty. At the moment, she doesn't think that she wants to get a tracheostomy in the future, but as she learns about technologies that make communication easier, she is keeping her mind open and may consider it as the need for it becomes nearer.
¶ Russell
Russell is 58 with limb-onset ALS, and progression feels relatively slow. He also has some digestive issues, which he believes may make the side effects of the disease-modifying drugs available to him too difficult to handle in his daily life. Instead, he is going to focus on minimizing stress on his body and maintaining his mental health. He understands that he may not live as long taking this approach, but he believes that he is striking the right balance.