Amytrophic Lateral Sclerosis, "Lou Gehrig's Disease"
Amyotrophic Lateral Sclerosis (ALS) aka “Lou Gehrig disease” is a devastating disease that causes motor neuron degeneration. It affects the motor neurons of the anterior horn cells of the spinal cord, the motor nuclei of the brain stem and the upper motor neurons of the cerebral cortex (Porth, C., 2005). This degeneration causes muscle weakness and eventually leads to muscle atrophy. Death is an inevitable outcome of the disease. Most people die within 2-5 years of symptom onset, although some may live up to 15 years. The disease affects people of middle age, generally afflicting people from 40-70 years old. There is no known cause of ALS, although there has been some indication of a genetic link in a small percentage of cases (about 10%) (Follin, Mill, & Munden, 2006).
Patients with ALS initially complain of asymmetrical weakness, cramping, and muscle fatigue (Follin, et al, 2006). Other early symptoms include muscle stiffness and fasciculations (twitching). These symptoms progress to more serious muscle complications such as difficulty breathing and swallowing difficulties that can lead to aspiration and pneumonia. The ability to speak is also compromised. These symptoms are caused by the death of motor neurons in the brain stem that control the respiratory muscles (Porth, C., 2005). Although ALS eventually affects almost all muscle control, it does not affect cognition or awareness. Therefore, although the person may lose the ability to speak, swallow, and breathe effectively, they are actutely aware of everything that is happening to them (Follin, et al, 2006). This is an especially disturbing reality of the disease.
Diagnosis of ALS is made using a variety of tests, as no one test is definitive for diagnosis. Electromyography is used to determine abnormal electrical activity in muscles, while muscle biopsy is used to see atrophic fibers characteristic of the disease. Diagnosis is also made by ruling out other diseases that may cause similar manifestations such as multiple sclerosis, myasthenia gravis, or muscular dystrophy (Follin, et al, 2006). Lung function and swallowing tests may also be done to see the extent of disease progression (NIH, 2010).
ALS has no known cure. The only FDA drug specifically approved to treat ALS is riluzole. This drug can prolong life by 3-6 months (Porth, C., 2005). It works by decreasing glutamate in the cerebral spinal fluid, which is thought to contribute to symptoms of ALS. Other drugs used to treat the symptoms of ALS include baclofen, diazepam and dantrolene to help control muscle spasms and amitriptyline to help control excessive drooling. Patients may also be prescribed antidepressants to help with depression associated with the diagnosis. (AAFP, 1999)
Nursing interventions for ALS include helping the patient perform ADLs and assisting with finding tools that will help the patient maintain his independence for as long as possible. As the disease progresses, the patient may require suctioning and mechanical ventilation (Follin, et al, 2006). Nurses can assist with ROM activities to help the patient maintain mobility for as long as possible. Because ALS is a terminal disease, it is important for the nurse to discuss the patient’s wishes regarding end-of-life care and interventions with the patient early in the disease process. The nurse should encourage the patient and family to discuss their feelings and assist them in coping with the diagnosis. Involvement in support groups should be encouraged. A list of support groups can be found at the ALS organization’s website at www.als.org. The ALS organization can also assist with locating adaptive devices that are available to loan from other members.
Follin, S., Mill, E., & Munden, J. (Eds). (2006). Diseases: A nursing process approach to excellent care. Philadelphia, PA: Lippincott Williams & Wilkins.
NIH (2010). Amytrophic Lateral Sclerosis. Retrieved from: http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001708/
Porth, C. (2005). Pathophysiology: Concepts of Altered Health States. Philadelphia, PA: Lippincott, Williams, and Wilkins.
Walling, A. (1999). Amytrophic Lateral Sclerosis: Lou Gehrig’s Disease. Am Fam Physician. 1999 Mar 15;59(6):1489-1496. Retrieved from: http://www.aafp.org/afp/1999/0315/p1489.html