In 1939,
baseball player Lou Gehrig’s career came to an end when
he became afflicted with an illness now known as “Lou Gehrig’s Disease”.
From that moment, the illness gained recognition internationally as a serious
condition, but it had been identified in 1869.
اضافة اعلان
As the neurons begin to degrade, so too does the individual’s ability to move until a voluntary movement ceases.
The official name
for this condition is amyotrophic lateral sclerosis (ALS) and was identified by
French neurologist Jean-Martin Charcot. In recent years, one notable figure in
the scientific community also afflicted with the condition was
Stephen Hawking. Hawking lived to be 76 years old, which is astonishing considering the
average survival time for ALS sufferers is two to five years. As more awareness and
research has been put into the study of ALS, the chances of survival and
longevity also increased.
May is ALS
awareness month; it is used as an opportunity to educate the public and bring
attention to the condition in hopes of helping improve the lives for those
affected.
ALS is a neurodegenerative disease in which the nerves in the body begin
to die or atrophy. ALS affects a specific type of neurons known as motor
neurons. Motor neurons relay signals from the brain, down the spinal cord, and
to skeletal muscles which initiates and controls muscle movement. As the
neurons begin to degrade, so too does the individual’s ability to move until a
voluntary movement ceases. As such, in Europe the disease is referred to as motor neuron disease (MND). Unfortunately, there is no cure for ALS, which is
progressive in nature.
Based on a 2019
review, worldwide, ALS is estimated to affect 4.42 people in 1 million.
Although a seemingly low prevalence rate, in the US, 5,000 people are diagnosed
with ALS every year. Furthermore, a 2016 study assessed the projected increase
of ALS from 2015 to 2040. The study used multiple countries around the world,
two of which were Libya and Iran, which belong to the
(MENA) region. Since Jordan has no study on the prevalence of ALS, these
two countries can help reasonably estimate the potential increase in cases
within the region. According to this study, the projected increase in ALS cases
is estimated to more than double in Libya and Iran (117 percent and 112
percent, respectively).
ALS symptoms are frequently divided into two categories: limb onset and
bulbar onset. They refer to where the earliest signs of ALS begin to appear and
predict the progression of the disease. Limb onset is the most common form and
occurs in 70 percent of those with ALS. For those with limb onset, symptoms
will begin to manifest in the arms or legs. In the arms, symptoms may include
weakness, stiffness, cramping, or loss of dexterity. In the legs, symptoms may
include difficulty walking, tripping, and a condition known as foot drop, which
occurs when the individual has difficulty lifting the front half of the foot
while walking. In the early stages of limb onset, symptoms only occur in the
legs or arms, but not both. Fortunately, limb onset ALS progresses more slowly
than other types.
Bulbar onset ALS is
less common and affects a portion of the brainstem known as the corticobulbar
area, which is responsible for many movements in the face. In those with bulbar
onset, common symptoms include difficulty talking, swallowing, and muscle
spasms in the face or throat. There are also some rare sub-types of ALS that
occur far less frequently; one is respiratory onset ALS, in which the muscles
that control breathing are affected first.
In the early stages of the
disease, symptoms may be relatively mild and
difficult to detect. Typically, there will be small, infrequent muscle twitches
in the mouth, throat, face, and limbs. Over time, the frequency of these
twitches will likely begin to increase. Additionally, it may become more
difficult to perform daily tasks, and symptoms may occur more on one side than
the other. As the condition progresses, symptoms typically begin to occur on
both sides and muscle weakness, weight loss, and muscle atrophy becomes more
common. By the late stages of ALS, total loss of voluntary movement will begin
to set in (paralysis), but sensations such as touch, sight, and hearing will
remain intact. It is also common to have bowel and bladder control affected in
these stages. Unfortunately, the most common cause of death for those with ALS
tends to be respiratory failure, as it becomes more difficult to breathe. At
the time of diagnosis, the typical lifespan is two to five years. However, 20 percent
of people live more than five years and 10 percent live for more than 10 years.
Although scientists and doctors have been aware of ALS for over a
century, many aspects of the cause for this condition are still unknown. To
date, there are two classifications of ALS that describe the origins of the
condition: sporadic and familial. Sporadic makes up the majority of cases and
means that there is no known cause. Familial means that the condition was
inherited from a parent and only makes up 5-10 percent of all cases. Certain
environmental factors are being investigated, but there is no definitive
evidence. What is known is that the risk of ALS increases with age and the
typical onset of symptoms occurs between the ages of 55 and 75. Additionally,
ALS is more likely to occur in males and in the white population.
Due to the progressive and debilitating nature of ALS, the condition is
associated with many serious, life-threatening complications that result in the
death of the individual. One of the most common complications of ALS relates to
speech and eating. As the individual loses strength and mobility in the mouth,
jaw, and throat, a host of problems begin to occur. Typically, it is common for
those with ALS to have difficulty speaking, but in severe cases, some may lose
the ability to speak entirely.
In addition to
speaking, eating also becomes increasingly difficult. ALS can affect the
person’s ability to chew and swallow, which poses the risk of choking.
Furthermore, due to the difficulty eating, those with ALS often experience
rapid weight loss and malnutrition. It is also believed that ALS may cause
individuals to burn more calories more quickly, which can increase weight loss.
One of the most
serious complications of ALS concerns the respiratory system. As the disease
progresses, breathing becomes more laborious and weakens. As a result, the risk
of pneumonia and respiratory failure also increases.
Although there is no cure
for ALS, there are many options to help those suffering. The main goal is to
help improve the quality of life, reduce symptoms, and delay the progression of
the disease. Support in the form of assistive devices can help improve mobility
and independence to a certain degree. Additionally, there are two current
medications approved for the treatment of ALS. They can help reduce symptoms
and even slow the progression of the disease, thus extending the lifespan.
However, it is best that the condition be treated as soon as possible to have
the best outcome. Being observant of the early signs for ALS can help ensure
you or a loved one receives treatment as soon as possible.
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