The chilling truth behind the cold challenge

Unless you have been living under a rock in the summer of 2014, you probably have seen
dozens of videos and pictures on the social media of various famous celebrities, charities and
CEOs getting themselves wet with ice-cold water from a bucket. So what is the reason behind
the ice bucket challenge? The goal is to raise awareness and donating for research in ALS
(amyotrophic lateral sclerosis). This great cause of pouring buckets of water had a tremendous
financial impact to support current researchPeople dumping ice water on their heads for a
cause have helped raise money over a hundred million dollars North America alone
(Organization). The act of doing the challenge is said to imitate how an ALS patient feels. For
that reason many might wonderwhat exactly is ALS?
Amyotrophic lateral sclerosis (ALS) or Lou Gehrigs disease is a progressive
neurodegenerative condition which causes neuron deaths in the brain and spinal cord. The
neurons are being affected are called motor neurons. They are part of the nervous system who is
responsible to deliver a response instruction from the spinal cord to the muscle when encounter a
stimulus. Soon after the muscles triggers an appropriate feedback as the leg extends. In all, they
deliver any sent impulses from the spinal cord as needed for any kind of muscle contractions in
both lower and upper body. Due to ALS, which damages the motor neurons, messages are stuck
at the spinal cord, it cannot be deliver to the muscles for proper feedback. When this condition
continues for a long period of time, the muscles are no longer capable of doing its job, they begin
to weaken and shrinkthis is known as muscle atrophy. The prognosis for many individuals who
have ALS is bleak after diagnoses due to ALSs properties as a progressive disease, eventually
the voluntary muscles such as the heart and lungs begin to weaken and shrink as well, resulting
in respiratory and cardiac failure. The disease is fatal within 3 years, moreover the life
expectancy of an ALS patient averages about 2 to 4 years (MDA).
The famous Yankees baseball player, Lou Gehrig, was the first professional athlete to
discover the disease in 1939 hence the name Lou Gehrig Disease. This rose more awareness and
attention in the medical field towards the research of ALS. Gehrig realized there was something
wrong with his body when he could not do simplest tasks such as shaving and using the fork.
These are the early symptoms of an ALS patient. They may experience weakness in a particular

limb, fasciculation (twitching of muscles), incapability of controlling facial expressions and

blemished speeches.
Although the direct cause for ALS hasnt been discovered, but researchers and scientists
have bits and pieces of clue which may indirectly cause ALS. They gathered the indirect causes
into 2 different categoriesfamilial genetics and sporadic. In familial genetics. Subjects have a
genetic predisposition to the disease with an inheritance of one or more faulty geneaccounts
for only 10% of the cases (Genetics). Additionally, the sporadic case is when subjects have no
family history of the disease, however could be diagnosed indirectly through protein
mishandling. This accounts for 90% of the cases (Genetics). In spite of no scientific evidences
towards protein mishandling, some researches still shown that they play a role. There are two
proteins that are considered as the primary suspects towards ALS. Ubiquilin 2 and Glutamate.
Ubiquilin 2 Is located in the spinal cord and the brain, its function is supposed to repair or
dispose damaged proteins within the neurons. However, in ALS patients, the Ubiquilin 2 is
unable to remove or repair the damaged proteins as they pile up in the nerve cells causing
blockage of transmission signals. The other suspect is Glutamate, it is a type of amino acid.
Moreover it is believed that prolonged glutamate in the brain are toxic to neurons.
Unfortunately today there is no cure for ALS, moreover there are treatments. The only
proven treatment against ALS effectively is the medication Riluzole. It reduces the damages of
motor neurons by decreasing the amount of glutamate. As I explained in the previous paragraph,
glutamate is toxic to the neurons. However Riluzole blocks the release of glutamate into the
brain. Thereby slowing down the progression of ALS. Other treatments are more focusing on
improvisation of individuals life after detected with ALS. Some of the improvisation includes
medical, financial and emotional needs. Physiotherapists contribute by handing out equipment
such as drivable wheelchairs, which can assist patients with mobility. Additionally, they can
prescribe medications to reduce fatigue and ease muscle twitching.
The research towards ALS took another big step in 2014 through the ice bucket
challenge funding, and still working hard to find new ways to cure or extend lives from the
epidemic. Some of the current research involve using the human embryonic cells. This theory is
called the cell replacement therapyusing human embryonic stem cells to create astrocyte
persecutors, a type of cell that support the proper functioning of neurons (neurotransmission and

neuron metabolism) (Mullen). The astrocytes persecutors are transplanted into the patients body,
soon after they will mature into healthy neurons inside (Mullen). Thereby reversing the problems
in ALS. Another research that is currently occurring towards the stemming of ALS is gene
therapy. Gene therapy is the ability to control genes and turning DNA on or off based on desire.
This could be a very big breakthrough for familial cases of ASL because those are caused by one
or more defected gene. If the faulty gene(s) can be replaced with a healthy one, that would
complete cure the familial cases of ALS. The last current research related to ALS is being able to
gain control of protein Ubiquilin 2. On the assumption that it can be controlled, the protein can
remove all of the damaged neurons from the brain and spinal cord, thus allowing
neurotransmission. This indicates the slowing down of ALS progression. Therefore prolong
lives.