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Article A: May 2024 Journal Club Article A
1A): Which diseases are generally evaluated and considered before ALS? (page 1370) (Feldman et al., 2022)
A) Small Pox B) Multifocal Motor Neuropathy with Conduction Block C) Axonal Motorpredominant Chronic Inflammatory Demyelinating Polyneuropathy D) Spinobulbar Muscular Atrophy
2A): While no cure for ALS, which two medications can be used (in some countries) for slowing ALS progression? (page 1373) (Feldman et al., 2022)
A) riluzole B) acetaminophen C) edaravone D) apixaban
Article B: May 2024 Journal Club Article B
1A): What type of modality is used to accomplish gene silencing? (Page 652, box 1) (Akçimen et al., 2023)
A) Mitochondrial Therapeutics B) Oligonucleotide Therapeutics C)Polycompositionaligonalitiocalitical-Phase B Therapeutics
Citations:
Article A: Feldman, E. L., Goutman, S. A., Petri, S., Mazzini, L., Savelieff, M. G., Shaw, P. J., & Sobue, G. (2022). Amyotrophic lateral sclerosis. The Lancet, 400(10360), 1363–1380. https://doi.org/10.1016/s0140-6736(22)01272-7
Article B: Akçimen, F., Lopez, E. R., Landers, J. E., Nath, A., Chiò, A., Chia, R., & Traynor, B. J. (2023). Amyotrophic lateral sclerosis: Translating genetic discoveries into therapies. Nature Reviews Genetics, 24(9), 642–658. https://doi.org/10.1038/s41576-023-00592-y
I asked Renee what kinds of diagnosis you have been seeing and I felt like I personally didn’t know a lot about ALS. I thought it was interesting in article B that they were using different therapies to just silence the gene that was causing the main issues. It just seems like something I never remember seeing on the floor and perhaps we never really see that as inpatient.
I agree with Charles regarding the ability to simply silence genes. It’s fascinating to me to learn about all the things that researchers are doing behind the scenes to help patients with ALS and other debilitating diseases.
I cared for the same patient Renee. These articles were definitely eye opening to see all of the research going on. I hope they can find a cure or earlier detection in the future. It is very sad and difficult to care for these patients as they become more medically complex and progress towards end of life.
Article A
1a: B,C,D
2a: A,C
Amyotrophic lateral sclerosis (ALS) is a rare and fatal neurodegenerative disease. The symptoms of ALS typically align with several other illnesses, thus diagnosis of ALS is typically delayed. The delay in diagnosis is unfortunate as it often results in rapid patient decline. I found it interesting that patients who present to their doctor with changes in cognition typically have a more rapid disease progression and survive for a shorter amount of time compared to those who originally have normal cognition. We recently had a patient on our unit that had ALS, but that was the first time I have seen a patient with this diagnosis during my nursing career.
Article B
1b: B
ALS affects motor neurons, thus resulting in progressive muscle weakness. ALS is a fatal disease with death typically occurring 2-5 years after the onset of symptoms. Some patients with ALS have a family history of the disease, but a large majority do not. Advances in research have helped pinpoint specific genes thought to be implicated with ALS. It is interesting to me that researchers have the ability to single out genes and are working on therapies to prevent these genes from malfunctioning. Treatment options are still limited at this time, but there is hope that more options are on the way, which would help assist us as nurses if we see more ALS patients in the future.
Article A
1a) b, c, d
2a) a, c
Amyotrophic lateral sclerosis is an incurable disease. There are some medications that help slow the progression although the disease it ultimately fatal. There is a lot of ongoing research into earlier detection which will be helpful for the future. Currently, diagnosis is often delayed. I recently cared for an ALS patient on our unit for the first time. It was interesting to read this article.
Article B
1b) b
I find it fascinating that they have been able to pinpoint cell mutations causing ALS. Despite being a rare disease, it is fairly well known after Lou Gehrig suffered from the debilitating disease many years ago. Some cases of the disease are genetic while others are spontaneous. Due to the high cost of personalized therapies, access is an issue. With expanding research of many neurological diseases, the future if hopeful for more personalized treatments and therapies. I am interested to see where this research leads in the future.
I agree Michele with high cost of therapies and whether or not people who are at high risk would even be able to test/pay for genetic testing to see if they have the genetic sequence.
1a) b, c, d
2a) a, c
1b) b
For the first article, I do not believe that I have taken care of a patient with amyotrophic lateral sclerosis though I and many of my co workers are familiar with taking care of neuro patients. After this article, I will be better able to care for these patients by better understanding the disease and options there are even if end of life planning is what the patient needs since there is sadly no cure. I now know that the disease itself is difficult to diagnose.
For the second article, new technology has proven genetic causes of ALS which is interesting and better for earlier intervention of the disease process to hopefully slow symptoms and help patients live longer.