eMedicine Specialties > Neurology > Neuromuscular Diseases
Amyotrophic Lateral Sclerosis: Follow-up
Updated: Jun 29, 2009
Follow-up
Further Inpatient Care
Inpatient care may be needed temporarily for patients with amyotrophic lateral sclerosis (ALS) who decompensate in the outpatient setting, for example due to pneumonia, or those who reach critical ventilatory failure without having appropriate ventilatory support in place and without having made end-of-life decisions (advance directives) declining such ventilatory support and electing comfort measures instead.
Deterrence/Prevention
Smoking is the only probable risk factor for ALS.25,45 Smoking avoidance may result in a decrease in age-specific incidence of ALS. However, if more patients at risk for developing the disease survive to each age due to reduced mortality from other smoking related diseases, such as cancer and cardiovascular diseases, this presumptive benefit of smoking cessation may not be realized fully. Furthermore, as the population survives to older ages (in which age-specific incidence of disease is higher) crude incidence of ALS may increase.
Individuals with a gene for FALS may benefit from genetic counseling if they wish to minimize the risk of transmitting the gene to the next generation.
Complications
- Progressive inability to perform activities of daily living (ADLs), including handling utensils for self-feeding
- Deterioration of ambulation
- Aspiration pneumonia
- Respiratory insufficiency
- Complications from being wheelchair-bound or bedridden, including decubitus ulcers and skin infections (While rare in patients with ALS, these complications can emerge if appropriate padding is not used.)
- Deep vein thromboses and pulmonary emboli (These complications are rare in patients with ALS, but have been encountered with greater frequency in the active treatment arm of some clinical trials.)
Prognosis
Patients with ALS survive on average 3 years from disease onset. Patients with some variants of the disease may have a longer course. Patients who are younger or with limb onset survive longer on average. The most effective predictor of survival is the rate of observed disease progression. Estimates derived as a ratio, where loss according to various measures serves as the numerator and time elapsed from disease onset to the time of measurement serves as the denominator may also provide more individualized prognostic information.
Patients the author has surveyed have indicated ambivalence about being offered individualized information early in the course of the disease (when it may matter most).85 The author does not offer patients individualized prognostic information when he sees them first. When patients do request it, he asks them to consider the possible implications of the answers they might receive and then they can ask him again at a subsequent visit.
Patient Education
- ALS Association, Living With ALS Manuals
- Muscular Dystrophy Association: MDA ALS Caregiver’s Guide
- Armon C. ALS 1996 and Beyond: New Hopes and Challenges. A manual for patients, families and friends. Fourth Edition. 2007. Published by the LLU Department of Neurology, Loma Linda, California.
- Doctor's Guide, patient resources: ALS (Lou Gehrig's Disease)
- ALS newsletter of the Muscular Dystrophy Association
- eMedicine's Brain and Nervous System Center
- eMedicine's patient education article Amyotrophic Lateral Sclerosis (Lou Gehrig Disease)
Miscellaneous
Medicolegal Pitfalls
- Patients are eligible for social security and Medicare benefits once they are diagnosed with ALS, without the waiting period required of other patients with chronic diseases. Patients should be advised to apply early.
- Since September 2008, ALS has been considered a service-connected conditions for US Veterans, and they are eligible for care and benefits. Eligible patients should be advised to apply early. Volunteers from local branches of veterans organization may be able to assist in preparing the application and moving it rapidly through the approval process.
- End of life issues may be discussed and clarified early. However, this may not work well for some patients. The physician should be aware of the individual state laws that regulate these issues, encourage, if appropriate for the patient, completion of advance directives, and document the patient’s preferences in the medical records, whether formal advance directives have been written or not.
- For patients with limited access to home-based resources, social service professionals may be able to assist with placement.
- Local branches of advocacy organizations (ALSA and MDA) may be excellent resources to inform patients of what is available to them locally.
- Patients will have decreasing ability to sign documents or to mobilize themselves for taking care of personal affairs. Some may benefit from legal advice. The earlier they do so the better, but they may need to recover from the initial shock of the diagnosis.
- All patients will become at risk for falls as the disease progresses: they should be informed and cautioned gently. Most patients tend to sustain several falls before they relinquish the independent activities that led to the falls.
- All patients will become unable to drive as their disease progresses. They need to be alerted. Most tend to be gracious about quitting before their driving results in injury. Some states require mandatory reporting by practitioners to the Department of Motor Vehicle Affairs.
Special Concerns
- Support groups are available to patients in many communities.
- Burnout of the primary caregiver needs to be anticipated and avoided by assuring that the primary caregiver is not the only caregiver.
- Patients with ALS may wish to help with the search for treatments for the disease through participation in clinical trials. They should be encouraged to focus on trials that have been listed with the ALS Association and with ClinicalTrials.gov, a registry of federally and privately supported clinical trials conducted in the United States and around the world.
- Patients often request prescription of available pharmaceuticals for off-label use in the hope of slowing disease progression. Since all such pharmaceuticals, so far, have either made patients worse when tested in double-blinded placebo-controlled studies or have had no benefit, this is not something that can be recommended.
More on Amyotrophic Lateral Sclerosis |
| Overview: Amyotrophic Lateral Sclerosis |
| Differential Diagnoses & Workup: Amyotrophic Lateral Sclerosis |
| Treatment & Medication: Amyotrophic Lateral Sclerosis |
Follow-up: Amyotrophic Lateral Sclerosis |
| Multimedia: Amyotrophic Lateral Sclerosis |
| References |
| Further Reading |
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Further Reading
- ALS Association, Living With ALS Manuals
- Muscular Dystrophy Association: MDA ALS Caregiver’s Guide
- Brown Jr, RH, Swash M, Pasinelli P, eds. Amyotrophic Lateral Sclerosis. 2nd Edition. Informa Healthcare, 2006.
- Mitsumoto H, Przedborski S, Gordon PH, eds. Amyotrophic Lateral Sclerosis. Taylor and Francis. 2006.
- Miller RG, Gelinas D, O'Connor P. Amyotrophic Lateral Sclerosis. AAN Press. Demos 2004.
Keywords
amyotrophic lateral sclerosis, ALS, Lou Gehrig disease, Lou Gehrig's disease, Charcot disease, Charcot's disease, motor neuron disease
Follow-up: Amyotrophic Lateral Sclerosis