Stop ALS! We too have a right to life!
Stop ALS! We too have a right to life!
The Issue
German | English
Hello,
My name is Johannes Kohner and two years ago at the age of 35 I fell ill with the rare amyotrophic lateral sclerosis, ALS for short. I am happily married and have two children aged seven and four. Now I have to communicate with the speech computer via eye control. My hands and legs no longer work and unfortunately eating is no longer possible due to the weakened tongue and swallowing muscles.
Jean-Martin Charcot discovered the disease over 150 years ago. And there hasn't been any major research breakthrough yet. While in cancer research there are multiple drugs to treat almost every type of disease, ALS can only be treated symptomatically, curing is currently not possible. Unfortunately. Every year well over 20,000 people in Europe contract ALS. And there will be more.
The physicist Stephen Hawkins was the most famous case of illness. He had the disease for 55 years, but also a very rare and slow variant of the disease. If the disease progresses normally, the average lifespan is only about two to five years.
In ALS, the motor neurons in the brain and spinal cord die. These nerve cells transmit the signal for movement to the muscles. If the nerves die, there are motor disorders up to the absolute loss of the movement function of the arms, legs, head and torso. Once the muscle-controlling nerves are gone, the body suffers massive muscle wasting. But the swallowing and breathing muscles are also less and less activated and eating becomes a torture, a PEG tube becomes necessary for survival over time, otherwise you would starve and die of thirst, but the risk of pneumonia is also very likely due to constant swallowing. Pneumonia is a common cause of death in ALS. Breathing becomes shallower and ventilation becomes necessary as the disease progresses. If breathing is affected, it also affects speech. At first it sounds drunk, but it becomes increasingly indistinct, to the point of total incomprehensibility. The psychological burden of verbal misunderstandings is increasing. With increasing disability, there is a risk of a relationship stress test that not every marriage, relationship and friendship can withstand. This disease destroys marriages and friendships on a large scale.
The psychological burden on the sufferer is extreme, so in just a few months/years you have to experience the loss of movement in all limbs, as well as speaking, swallowing and finally breathing, even if you are in a clear and full mental state. But the psychological burden on the closest relatives is, in my opinion, many times higher, it's close to going crazy when you have to watch your loved one's physical decay and can't do anything about it.
Now there is finally hope, the result of a phase 2 study published in May 2022 by the Swedish company Tikomed has been shown not only to slow down the progression of the disease, but even to stop it.
Therefore, on behalf of all ALS sufferers, I call on the German Bundestag, the EU Commission, the Swedish Ministry of Health to contact the European Medicines Agency (EMA) and its Committee for Medicinal Products for Human Use (CHMP) so that Article 83 of Regulation (EC) No 726/2004 is used and the drug ILB, which is currently in the study, receives Compassionate Use Status from the Swedish pharmaceutical company Tikomed. The drug needs of people suffering from ALS are not covered at all. Riluzole, the only approved treatment in the EU, increases survival by approximately 3 months. There is an absolute need for action in this regard. The EMA holds itself responsible for its stubborn and ignorant attitude towards ALS sufferers. The many tens of thousands of people condemned to death in Europe must finally be helped. Here is the link to Compassionate Use.
https://www.ema.europa.eu/en/human-regulatory/research-development/compassionate-use
The policy demands the following:
- Rapid approval of the drug ILB
- EU-wide access to the drug ILB for all ALS patients
- A promise of treatment also for the long-term sick
- Elimination of bureaucratic hurdles
- An acceleration of research
- Good financial conditions for future production
- A rare disease awareness campaign in the media
- A fixed funding pot to increase the attractiveness for future studies
The recent past has shown us all that research can be faster and that the approval process does not always have to last forever. The clock is ticking...
To promote the petition, the Ice Bucket Challenge2.0 was launched in May 2023. You can follow the link below.
http://tiktok.com/@icebucketchallenge2.0
You can see the horror of this terrible disease in the following video from the ALS Association in Canada. This hits the topic exactly and that's exactly how it works.
169,096
The Issue
German | English
Hello,
My name is Johannes Kohner and two years ago at the age of 35 I fell ill with the rare amyotrophic lateral sclerosis, ALS for short. I am happily married and have two children aged seven and four. Now I have to communicate with the speech computer via eye control. My hands and legs no longer work and unfortunately eating is no longer possible due to the weakened tongue and swallowing muscles.
Jean-Martin Charcot discovered the disease over 150 years ago. And there hasn't been any major research breakthrough yet. While in cancer research there are multiple drugs to treat almost every type of disease, ALS can only be treated symptomatically, curing is currently not possible. Unfortunately. Every year well over 20,000 people in Europe contract ALS. And there will be more.
The physicist Stephen Hawkins was the most famous case of illness. He had the disease for 55 years, but also a very rare and slow variant of the disease. If the disease progresses normally, the average lifespan is only about two to five years.
In ALS, the motor neurons in the brain and spinal cord die. These nerve cells transmit the signal for movement to the muscles. If the nerves die, there are motor disorders up to the absolute loss of the movement function of the arms, legs, head and torso. Once the muscle-controlling nerves are gone, the body suffers massive muscle wasting. But the swallowing and breathing muscles are also less and less activated and eating becomes a torture, a PEG tube becomes necessary for survival over time, otherwise you would starve and die of thirst, but the risk of pneumonia is also very likely due to constant swallowing. Pneumonia is a common cause of death in ALS. Breathing becomes shallower and ventilation becomes necessary as the disease progresses. If breathing is affected, it also affects speech. At first it sounds drunk, but it becomes increasingly indistinct, to the point of total incomprehensibility. The psychological burden of verbal misunderstandings is increasing. With increasing disability, there is a risk of a relationship stress test that not every marriage, relationship and friendship can withstand. This disease destroys marriages and friendships on a large scale.
The psychological burden on the sufferer is extreme, so in just a few months/years you have to experience the loss of movement in all limbs, as well as speaking, swallowing and finally breathing, even if you are in a clear and full mental state. But the psychological burden on the closest relatives is, in my opinion, many times higher, it's close to going crazy when you have to watch your loved one's physical decay and can't do anything about it.
Now there is finally hope, the result of a phase 2 study published in May 2022 by the Swedish company Tikomed has been shown not only to slow down the progression of the disease, but even to stop it.
Therefore, on behalf of all ALS sufferers, I call on the German Bundestag, the EU Commission, the Swedish Ministry of Health to contact the European Medicines Agency (EMA) and its Committee for Medicinal Products for Human Use (CHMP) so that Article 83 of Regulation (EC) No 726/2004 is used and the drug ILB, which is currently in the study, receives Compassionate Use Status from the Swedish pharmaceutical company Tikomed. The drug needs of people suffering from ALS are not covered at all. Riluzole, the only approved treatment in the EU, increases survival by approximately 3 months. There is an absolute need for action in this regard. The EMA holds itself responsible for its stubborn and ignorant attitude towards ALS sufferers. The many tens of thousands of people condemned to death in Europe must finally be helped. Here is the link to Compassionate Use.
https://www.ema.europa.eu/en/human-regulatory/research-development/compassionate-use
The policy demands the following:
- Rapid approval of the drug ILB
- EU-wide access to the drug ILB for all ALS patients
- A promise of treatment also for the long-term sick
- Elimination of bureaucratic hurdles
- An acceleration of research
- Good financial conditions for future production
- A rare disease awareness campaign in the media
- A fixed funding pot to increase the attractiveness for future studies
The recent past has shown us all that research can be faster and that the approval process does not always have to last forever. The clock is ticking...
To promote the petition, the Ice Bucket Challenge2.0 was launched in May 2023. You can follow the link below.
http://tiktok.com/@icebucketchallenge2.0
You can see the horror of this terrible disease in the following video from the ALS Association in Canada. This hits the topic exactly and that's exactly how it works.
169,096
The Decision Makers
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Petition created on 5 January 2023