Why is SLA (ALS) a severe incurable disease?

In this post I would like to present to you a very serious, incurable disease that is SLA.
I describe it because many of my patients are in contact with this disease.
Amyotrophic lateral sclerosis
sclerosis lateralis amyotrophica

Lou Gehrig (1903-1941)
Lateral amyotrophic lateral sclerosis (Charcot's disease, Lou Gehrig's disease, motor neurone disease, Latin sclerosis lateralis amyotrophica, SLA, amyotrophic lateral sclerosis - incurable, progressive neurodegenerative disease that leads to destruction of the anterior spinal cord cells, cranial nerve nuclei of the medulla and neurons of the pyramidal pathway, i.e. selective damage to the peripheral (lower) and central (upper) motor neuron.

The disease was first described by Jean-Martin Charcot (1825-1893) together with Alexis Joffroy (1844-1908). In the United States, the disease is commonly known as Lou Gehrig's disease (Lou Gehrig's disease) from the name of the famous American baseball player Henry Louis Gehrig, who died of ALS in 1941.

It appears mainly in the 6th and 7th decade of life, more often in men. The incidence of the disease is estimated at 2-4: 100,000. The disease may also occur in young people, although much less frequently (1-3: 500,000).

Symptoms and course of the disease:

The first symptoms are muscular atrophies related to short hand muscles, spastic paresis of the lower limbs and sometimes bulbar features. In the further course there are lapses of subsequent muscle groups with characteristic fasciculations and progression of spastic symptoms. In patients during the development of the disease, there is a slow but systematic deterioration of physical fitness, and in later stages to complete paralysis and eventually death by stopping the work of the respiratory muscles. It is generally accepted that ALS leads to death within 3-5 years; the most common death due to ALS is due to respiratory failure (respiratory muscle paralysis). Apart from few cases, ALS does not touch the intellectual sphere of a human being, an example is astrophysicist Stephen Hawking, who fought the disease for over 50 years, and American guitarist Jason Becker, who during the disease gave two discs, fighting disease from the age of twenty.

Treatment:

There is no effective treatment for causal disease so far. Riluzol (Rilutek) may prolong survival and / or delay the onset of respiratory failure in ALS patients, slowing down the progression of the disease. In controlled clinical trials, the average survival was prolonged by approximately 2-3 months [4].

Palliative treatment
(Reducing the discomfort of the disease)

Replacement breath: patients are placed on the respirator.
Palliative feeding therapy:
when masseter paralysis occurs - chewing gum disorders: complete, balanced liquid diets (Ensure Plus, Ensure Plus HN, Enaure) served with a spoon or drinking straw.
when swallowing disorders occur: gastrostomy feeding (preferably PEG) or by gastrojejunostomy - PEGJ using preparations such as Jevity
Palliative diet therapy targeted
Targeted nutrition therapy is aimed not only at solving the nutritional problems of the patient. In the case of SLA, it is intended to facilitate mechanical ventilation. The use of low-respiratory diets is recommended.

Induced pluripotent stem cells
ALS patients are iPSC donors, from which (up to 2011) more than 30 cell lines have been obtained, which can serve as a model of the disease, so they are used to better understand the causes and course of the disease.

I provided a source from Wikipedia

More info :http://www.alsresearchforum.org