Amyotrophic lateral sclerosis
I was recently challenged to participate in the latest viral social behavior: the ice bucket challenge. The activity raises awareness for Amyotrophic lateral sclerosis, now more commonly known as ALS. I have decided to respond to the challenge with this blog post rather than pouring water over my head, since I’ve come to realize that watching over twenty ice challenge videos has taught me next to nothing about the disease!
ALS is both the most common and most aggressive type of adult motor neuron degeneration. The death of upper motor neurons in the motor cortex and lower motor neurons in the brainstem and ventral horn of the spinal cord result in a progressive loss of muscle control. This progression is characterized by stiffness, overactive reflexes, muscle twitching, muscle atrophy, and finally full paralysis. Approximately 15% of patients also suffer from cognitive and behavioral problems known as frontotemporal dementia due to the death of neurons in the prefrontal and temporal cortex in the brain[1,5].
Finally, it is a heterogeneous disease: the age, site of onset, rate of progression, and the presence and degree of cognitive dysfunction can vary widely between patients. The disease is fatal within 3-5 years of onset.
No other disease has captured the modern imagination with such sensational horror as Ebola. The possibility of infection being transported into dense cities and the western world along with the possibility of bioterrorism captures our attention. However, much of the disease’s infamous reputation is due to sensationalized stories of the outbreaks along with the popular perception of the disease causing a disconcerting amount of bleeding from every orifice. In actuality, the disease’s outbreaks are relatively rare (although increasing) and the presentation itself only includes external bleeding in less than half of the cases. Still, with a mortality rate ranging between 40-90% and no current treatments, Ebola is not a disease to be taken lightly.
No one really likes vaccinations. Children view the procedure with the trepidation of a victim being pursued by a madman with a butcher’s knife, while parents make soothing, inane comments to their terrified offspring, such as “the sharp, pointy weapon is nothing to be afraid of!” as they are forced to pry their children from underneath the doctor’s table (my sister was particularly prone to this type of needle-avoidance). Personally, I view my own shots with a perverse fascination, but that probably comes from being, as my parents say, ‘an unusual child.’ In general, vaccinations are viewed by both parents and patients with resigned acceptance, although in the past decade there has been an upsurge of media attention toward possible side effects. Some individuals have become leery of the procedure, choosing to keep their children unvaccinated. Just how much of these concerns are based on truth, and how much stems from misinformation?
It is important to understand the science behind vaccinations, and the process of development, before jumping to conclusions. Vaccines have provided relief from decimating diseases, even eradicating mass killers such as smallpox. They are an unparalleled scientific miracle, which utilizes the wonders of our own immune system to provide protection from deadly diseases.
Leprosy is known as the Death before Death; a disease of unparalleled historical stigma and fear that has earned the appellation kushtha, ‘eating away.’ Today, whilst its effects are none the less awful, it is known under the more innocuous title of Hansen’s Disease. In 1948, Dr. Earnest Muir wrote that leprosy was the most dreaded disease—ironically, not because it kills, but rather, because it leaves its victims alive. Leprosy and humans have a long, bitter relationship. Though it has plagued us since at least 2000 BC, we barely understand it four thousand years later and we are still wrestling with its legacy of stigmatization.