What actually came of that ice bucket challenge?

During the ALS Association campaign, participants were challenged to dump buckets of ice water over their heads, while filming the scene, and then donate 10 dollars to the ALS Association. Participants would then also invite 10 other friends to do the same, and those who didn’t accept the challenge were requested to donate 100 dollars. By covering their bodies with ice cold water, the participants, for only a few seconds, experienced the feeling of muscular paralysis, to better understand ALS patients. Within the first eight weeks, the ALS Association was able to raise 13 times more donations than they had in the entire year before; all because of the media attention that this campaign received. Therefore, there is no doubt about the positive effect this campaign had on research projects for this disease. Many criticized the campaign for focusing too much on celebrities instead of actually discussing the facts and highlighting the disease.

An amyotrophic lateral sclerosis is a neurodegenerative illness which affects the first and second motoneuron. Motoneurons are nerve cells which are involved in neuronal connections. The first motoneuron lies in the motor-driven cortex, an area responsible for planning and executing random movements. Its extensions are connected to the second motoneuron, which is located in the spinal marrow. These neurons are also known as alpha motoneurons, responsible for sending nerve impulses to the individual muscles. If motoneurons are damaged, the brain can no longer transmit nerve impulses to the muscles, resulting in muscle paralysis.

During ALS, motoneurons degenerate. Scientists have not yet been able to understand why this happens, though. To this day there are no therapies for treating the causes, leaving ALS incurable. Every year, approximately 2 to 3 people in every 100,000 are affected by ALS. The disease often sets in between the ages of 60 and 80. The first parts to become impaired are usually the small muscles in the hands and fingers, as well as the shoulder and calf. After hitting, the disease then spreads out to all muscles, leaving the patient impaired, even in swallowing and breathing. If not given artificial respiration, many can die from respiratory insufficiencies within two to five years. It is hard to determine exactly how quickly the disease will affect a person. Stephen Hawking, for example, has been suffering from ALS for over 50 years, which is a very slow case of the disease.

Two years after the ice bucket challenge in 2014, ALS is still not curable, but at least researchers have been able to progress in the area – even if in small steps. In the summer of 2015, at the beginning of the second round of the ice bucket challenge, scientists from the John Hopkins University School of Medicine published their study results on the protein TDP-43 and its functions – at least in animal models – and how this protein is responsible for reading the DNA within cells. Years before, the TDP-43 was deemed a responsible factor for the ALS-typical protein accumulations inside the degenerating cells and seemed to be involved in the development of ALS.

This summer, a gene was identified, which appears damaged in many ALS patients. The so-called NEK1 gene is possibly in charge of protecting DNA from spontaneous damage. Many ALS patients exhibiting mutations within this gene could possibly offer a causal correlation to the disease. Even with these discoveries, there is much research yet to be done on the topic. ALS patients, in many cases, remain dependent on supportive measures that only help ease the symptoms, not the cause. At some point, people affected by ALS will still require a wheelchair and cannot go without respiratory support or help with eating and communicating.

The damaged motoneurons lead to paralysis and amyotrophia. In the course of the disease, many patients develop a dysarthria, where they can no longer speak, as well as a dysphagia, where they can no longer swallow. The dysphagia can lead to food accidentally being breathed in instead of swallowed, transporting it into the lungs and causing a lung infection. Eating becomes impaired and patients begin losing weight. This, in combination with the metabolic changes caused by the illness, can even cause pathological weight loss, respectively cachexia. A cachexia is most likely to worsen the ALS progression and should therefore be recognized very early on. For this reason, determining the body mass index (BMI) and regularly measuring the patient’s weight is an obligatory procedure for ALS patients. As an ALS patient’s mobility is strongly impaired, especially in the later stages of the disease, many patients will only be able to move around in wheelchairs. This makes regular weighing difficult. For patients in wheelchairs, seca offers a variety of wheelchair scales. The seca 677, for example, can hold up to 300kg and is equipped with a large platform that can be folded up and therefore stowed away easily. This device can calculate the BMI and transmit the data wirelessly to a seca printer or computer.

Aside from regular weight exams, the German Society for Neurology (DGN) recommends measuring the patient’s body composition by using of the bioelectrical impedance analysis (BIA). The seca mBCA is a medically validated BIA device that can quickly and precisely determine body compositions. It can distinguish between individual body compartments and deliver even more precise data about the patient’s nutrition status than what pure weighing or the BMI would do alone. A cachexia can therefore be recognized and treated early on.

Regularly examining an ALS patient’s nutrition status is essential and can offer insights into a developing malnutrition in its early stages. This aspect of an exam is an important pillar in optimizing the ALS patient’s individual therapy, as well as a better prognosis and quality of life.