Intended Audience: Pharmacists and managed care professionals.
Release date: August 16, 2018
Expiration date: August 16, 2019
Estimated time to complete activity: 3.0 hours
Type of activity: Application
Medium: Print with Internet-based posttest, evaluation, and request for credit
Amyotrophic Lateral Sclerosis: Disease State Overview
Amyotrophic lateral sclerosis (ALS) is a disease that results in the progressive deterioration and loss of function of the motor neurons in the brain and spinal cord, leading to paralysis. ALS affects approximately 16,000 individuals, with a prognosis for survival of 2 to 5 years. There are 2 types of ALS differentiated by genetics: familial and sporadic (idiopathic). Diagnosis is determined by excluding other conditions and utilizing clinical examinations, laboratory tests, and nerve conduction/ electromyography studies. Due to the collection of information from the participation of patients with ALS in registries, biomarkers and genes associated with ALS have been discovered. The best practices for the management of ALS include an interdisciplinary approach aimed at addressing the physical and psychological needs and desires of patients and their families and caregivers.
Disease-Modifying Treatment of Amyotrophic Lateral Sclerosis
Currently, there is no cure for amyotrophic lateral sclerosis (ALS) and the foundation of ALS management revolves around symptomatic and palliative care. Early diagnosis offers the best prognosis for a longer, quality life while living with the disease. Many medications are used to relieve symptoms but there are only 2 pharmacologic agents indicated for the management of ALS. For 2 decades, riluzole had been the mainstay of disease-modifying therapy, but in 2017, edaravone became the second agent approved in the management of patients with ALS. The mechanism of either agent is not well known. Riluzole is thought to reduce damage to motor neurons through an inhibitory effect on glutamate release, while edaravone is thought to act as a neuroprotective agent that prevents oxidative stress damage as a free radical scavenger. With the lack of treatment options, it is imperative for healthcare professionals to understand the nuances of using these 2 agents to optimize therapy and quality of life for patients with ALS.
ALS Managed Care Considerations
As a chronic neurological disorder characterized by the progressive deterioration of neuromuscular function, amyotrophic lateral sclerosis (ALS) renders significant physical, psychological, and emotional tolls on patients, their families, and caregivers. Coupled with and contributing to the severe impact on quality of life are the substantial economic costs, which can be direct and indirect, and personal as well as societal. Understanding the magnitude and specifics of the costs accompanying ALS will provide useful perspectives to pharmacists who treat patients with ALS.
Which one of the following is a gene associated with ALS development?
B) Urinary p75
What is the presumed mechanism of neuroprotective action of edaravone?
A) Voltage-gated Ca2+ channel blocker
C) SOD1 inhibitor
With such a debilitating disease as ALS, early diagnosis can be beneficial in a number of ways. Which of the following is a potential benefit to early diagnosis of ALS?
A) Reduced risk for tracheostomy
B) Increased cure rate
C) Extended time to medication initiation
D) Reduction in overall healthcare costs
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