“Recently, the disease is getting younger”: the head of the “Live Now” Foundation - about patients with amyotrophic lateral sclerosis

"Mysterious Disease"

- In your opinion, what do people who have never experienced it need to know about amyotrophic lateral sclerosis?

Are there any myths about ALS that are long overdue for dispelling?

- This is a disease that deprives a person of the ability to speak, move, breathe and swallow independently, while leaving the vast majority of patients with intact intelligence.

A vivid example is Stephen Hawking, who lived with this disease for more than 50 years, while actively traveling, making discoveries, teaching people.

Many athletes and scientists who have encountered ALS have worked to the last.

Because, probably, one of the most serious stereotypes about the disease is that a person allegedly turns into a vegetable.

Although in reality it is not.

This myth is based on fear.

Our society still reacts painfully to people with disabilities.

This is partly due to the almost complete lack of an accessible environment, partly to post-war stereotypes about the crippled, who were perceived as subhuman, and with many other factors.

People, especially in small towns, believe that from the moment of diagnosis, their life is over, doctors, social workers will never come to them, and if they live on the fifth floor without an elevator, then the patients themselves will never go outside again.

Hence, by the way, a very large number of severe depressions in people with ALS.

In addition, since the “classic” age of ALS onset is considered to be 50–55 years (although the disease has been getting younger lately, and scientists still don’t know why), we are usually talking about people who have long been formed, have lived for some life, the way of which they are forced to change dramatically.

But stroke or severe forms of cancer can also lead to loss of mobility, they also force a complete lifestyle change, and these diseases are more common than ALS.

I see the uniqueness of amyotrophic lateral sclerosis as an aggravating factor.

It is a rare disease, the cause of which is still unknown, although the first mention of ALS in medical books dates back to 1874 and research has been going on for decades.

There are no two identical patients - even if, for example, the hand began to suffer first, further development, its pace, etc. will differ.

That is why it is so difficult to diagnose.

There is still no answer as to why ALS is spreading so evenly around the world.

That is, it is not possible to find the influence of climate, nutrition, solar activity, even social status, as happens with many other diseases.

Perhaps it is because of all these mysteries that ALS remains such a challenge for the entire scientific community.

• © Live Now Foundation

- They say that this disease is often found in athletes, is it true?

- There are a number of studies that have shown that ALS is slightly more common in military and professional athletes.

Italian scientists even found out that strikers are more susceptible to this disease among football players.

There have also been studies that have linked the onset of ALS to smoking, brain injury, and gender—it is commonly believed that men are more likely to get sick.

However, it is important to emphasize that in all cases it was about such small indicators that some experts say that this can be regarded as an error.

That is, not, conditionally, 30% of athletes are prone to ALS, but from tenths to 2-3%. 

Nevertheless, patients still try to find some patterns.

Therefore, patient chats (and about 50 new families come to us every month) are seething with the same questions.

“Who had a concussion?

And who worked with chemicals?

Who was under severe stress?

They are constantly looking for the answer to the question "Why?"

and cannot find it.

This adds even more anxiety.

- Some relatives of patients compare ALS with spinal muscular atrophy: these are severe neurodegenerative diseases with expensive therapy, while SMA is still diagnosed quite quickly, and it is rather difficult to determine that a person has ALS.

Is it so?

- The difficulty is that both diseases are similar in manifestations.

But if SMA is a genetic disease and it is enough to make an appropriate analysis, then in the case of ALS there is no analysis or study that could unambiguously confirm it.

Less than 10% of patients have a familial mutation in the gene, and its presence does not mean at all that a person will develop the disease.

Taking a genetic test for ALS is pointless.

If we are talking about non-genetic forms of ALS, then diagnosis is carried out either by excluding other diagnoses, or, unfortunately, when the deterioration is already so strong that there are no other options left.

In the first stages, the symptoms of the disease are often mistaken for the consequences of a stroke, microstroke, intervertebral hernia.

For example, Fernando Ricksena, a famous football player who played at Zenit, who had access to the best doctors in the world, was operated on, believing that it was all about hernias.

And only after the operation, when ALS began to progress strongly, he was diagnosed correctly.

It is possible to detect the disease at an earlier stage, for this electromyography is performed.

But in Russia there are very few highly qualified specialists in this field.

We give lectures, present educational materials, in general, we are actively working to improve the diagnosis of neuromuscular diseases.

In terms of money, the prices are certainly lower than for SMA drugs, but they are still out of reach for most patients.

Everyone needs at least a NIV device (non-invasive ventilation of the lungs. - 

), an expectorant, an electric bed and a lift, that is, about 2.5 million rubles.

Our foundation accompanies thousands of families where someone is ill with ALS.

And according to our assumptions, there are at least 15 thousand such families in the country.

• © Live Now Foundation

"We work with passion"

- Do you somehow interact with state medical institutions?

“We have invested a lot of resources over the past five years in raising awareness about amyotrophic lateral sclerosis, including among doctors.

We agree on cooperation with the ministries of health, launched several serious educational programs, conducted a study in four regions, why it takes a long time to diagnose and at what stage the state system generally loses sight of a patient with ALS.

We cooperated with many ministries of health in terms of equipment: they told us which equipment is suitable for which categories of patients, how to use it.

We have a unique school of respiratory support: we conducted it not only for ALS, but in general for all nosologies where home ventilation may be required.

During the pandemic, these skills came in very handy: we were able to prove that it is not necessary to put a tracheostomy on a patient and injure him additionally, mask ventilation in many situations does an excellent job with the necessary tasks.

And it's great that resuscitators have now begun to use a non-invasive method of respiratory support for the patient.

— How did the fund survive the

“At the beginning, we faced a shortage of non-invasive lung ventilation devices.

Some wealthy people simply bought this technique home for themselves just in case.

Now we do not feel the lack of equipment.

Procurement, which was planned in a number of regions where we helped with consultations, is proceeding as usual and without delay, as far as I know.

— Has the work of the fund changed in any way since February 24?

- Of course, now the whole field of charity is very difficult.

But we faced the problems that are common now even before others.

We had a task to change the site for a long time, and in December we finally launched it.

But when restarting, the payment system began to issue endless errors, in connection with which both one-time donations and regular ones began to fall off.

And it is clear that a person tried to transfer money once, the second, the third - well, he will go to the fund, where there are no such failures.

As a result, by mid-February, we defeated these technical difficulties.

At the end of the month, for obvious reasons, all foreign banks were disconnected from transfers, and we had quite a lot of donations from individuals from abroad.

Further, a number of partners - legal entities with whom we had an agreement on educational programs (who acted as our sponsor) announced that they had withdrawn their budget for revision.

As a result, all transfers from legal entities came to naught.

• © Live Now Foundation

With great difficulty we closed April, but in May we had to fire five nurses.

For us, this was a terrible loss, given that we provided nurses for the most difficult patients or lonely people, as well as those where an elderly and no less sick person cares for the patient.

But even after that, we didn’t have enough money to pay all the due taxes, so I donated funds from my personal credit card to the budget.

In general, the situation with donations has not changed much.

We are working for the second month rather on enthusiasm.

It is impossible not to admire our team - no one leaves, everyone holds on, they continue to believe that everything will work out.

I don't want to stop our unique educational programs, which give very good results and are getting more and more demand from doctors.

- What are these programs, except for the respiratory one that you mentioned earlier?

— For example, we are holding the only conference on ALS in Russia, it is intended both for patients and, in recent years, for the interested medical community.

Created regional conferences: both scientific and practical.

We go to the regions for a couple of days, talk about the neurological aspect, palliative, respiratory, patronage care, show how to set up the device, consult doctors, what equipment is needed, how to work with such patients.

When I first came to this sphere, I was struck by the fact that in the 21st century in a civilized country, in a city of many millions, a person can die of hunger and thirst.

Because patients with ALS are becoming increasingly difficult to swallow, but they did not get a gastrostomy in a timely manner.

In Russia, in general, these devices are used very rarely, moreover, if they are installed, then most often this tube 20 mm in diameter is placed under general anesthesia, through a large incision.

That is, a difficult operation, after which it takes a long time to recover, and for a person with ALS, general anesthesia means that he will forever remain tied to a ventilator.

There is an alternative, but at that time there were practically no relevant specialists.

So we launched an endoscopic school where we train doctors to install a gastrostomy with minimal harm to the patient.

After the operation, a person can go home in 15 minutes.

In general, we hope that each region will eventually have at least one specialist who can work with children and adults and, if possible, use less invasive methods.

"The only successful flash mob"

— How can people express solidarity with ALS patients and their families?

What do the foundation and its beneficiaries need?

- Of course, like all charitable foundations - albeit very small, but regular donations that allow you to plan the work of the organization.

Plus, no matter how frivolous it may sound, support in social networks.

All these likes, shares, comments mean that more people will see the information and may want to help.

Unfortunately, often even the patients themselves, who are directly interested in the fund helping them, who constantly sit in patient chats, resist requests to be more active in social networks.

We do not ask you to publish your photo and medical history!

Just one like.

And two is better.

• © Live Now Foundation

- Probably the most famous action in support of ALS patients is the

— Marketers agreed that the Ice Bucket Challenge is the only truly successful flash mob in the world.

The action started in 2014.

Our fund did not exist then, and many of our fellow citizens later recalled that they could not find an organization that would help such people in Russia, so they sent donations abroad.

Then, with the money raised, they launched, among other things, the largest genetic study of ALS.

However, the vast majority of people perceived this whole flash mob as entertainment, such a comic test of courage, given that the action reached its maximum coverage in the summer.

According to polls, many participants or viewers of the action did not catch the essence.

The idea was to pour a bucket of ice water over yourself.

At this moment, any person from the temperature difference intercepts his breath and paralyzes him for a second.

Thus, a healthy person can momentarily experience what it is like to live with ALS, unable to breathe and move.

Since then, actions have been regularly held in different countries in support of ALS patients and for greater public awareness.

In the Netherlands, they organize a charity swim, in which even members of the royal family take part, and in Australia, stadiums of 6-10 thousand people gather for shares, walking marathons.

But it must be admitted that no one has succeeded in surpassing the Ice Bucket Challenge in terms of scale and virality, not only in the field of ALS, but also in other areas.