Cystic fibrosis is a genetic disease, in which the normal functioning of the mucous membranes is disrupted - this primarily affects breathing and digestion. In most European countries, the average life expectancy of a person with this disease is just over thirty years. In Russia, the figure is lower - twenty two - twenty five.
Taisiya Sheremet launched the #LetMneBreathe flash mob in support of people with cystic fibrosis - she wants to ensure that people with this diagnosis in Russia have access to quality drugs that can improve their standard of living. We talked with Taisia about life with cystic fibrosis and her activism.
About cystic fibrosis
As a child, I was with an ophthalmologist, who, without being too shy, asked my mother: "Do you have a disabled child?" - I was seven or ten years old. So I found out that I have a disability. I knew that my disease was called cystic fibrosis, but my parents did not say what it meant - apparently, they did not want to upset. I didn't know it was fatal. One summer I was at my grandmother's, and it occurred to me to Google cystic fibrosis. When I found out what it was fraught with and how it could end, I felt very bad. For some time I went to a psychologist - this is the only thing that helped me to cope.
Cystic fibrosis is a genetic multiple organ disease (affects several different organs. - Approx. ed.). Every twenty-fifth European has a specific gene breakdown. If two carriers meet and have a child, twenty-five percent of the time it will be a person with cystic fibrosis.
This disease disrupts the transport of chlorine ions in the body, and as a result, biological fluids become thicker. Because of this, the ducts of the pancreas become clogged: it ceases to secrete enzymes, food is not digested. The digestive system suffers, dystrophy develops, fever may occur, the liver and kidneys work with difficulty. Since the phlegm in the lungs is very viscous, bacteria grow in it; the alveoli burst, bleeding occurs, the temperature rises - symptoms are similar to that of extensive bronchitis. The volume of the lungs decreases, and the load on the heart increases: it begins to beat faster to compensate for the lack of oxygen. I have a mixed form of cystic fibrosis - problems with the lungs, and with the digestive system, and with the pancreas. The disease is difficult.
I always say that I am not the most exemplary patient. Not because I don't want to live
or I don't watch my health well - it just happens that way
We breathe air - it contains bacteria, viruses, mold spores and other pathogens. It is unlikely that in a person without disease it will settle in the lungs: the sputum that is constantly produced in them, in a normal situation, leaves the body by itself. But the viscous sputum of a person with cystic fibrosis accumulates, which means that bacteria have more breeding ground at their disposal. I need to do inhalations every day to thin the phlegm and drainage to artificially remove it from the lungs. These are special breathing exercises - you need to force yourself to cough with a strong exhalation. You may need courses of antibiotics, antifungal agents. At the same time, even if I lie with a fever and a temperature of forty, you cannot get infected from me - unless, of course, I have the flu.
Basic human metabolism refers to how many calories we burn at rest. The average person breathes in and out fifteen times per minute, and we are thirty - twice as often. People with cystic fibrosis have a very high metabolism and lose weight quickly due to a constant calorie deficit. We spend more energy and receive less.If a person without disease consumes two thousand calories per day, then we need four to five. This imposes restrictions on the diet - you need to eat balanced, but a lot to gain weight, although it is not very pleasant to overeat constantly. I eat six large meals a day. At the same time, you need to watch out for sweets so as not to develop diabetes mellitus, and the harmful effects of some foods are felt more strongly. People with cystic fibrosis are constantly taking antibiotics, which affects the liver, so you can't load it with alcohol either. And since the ducts of the pancreas are clogged, you need to additionally take enzymes in tablets so that this huge amount of food is digested and absorbed.
It takes a lot of time and attention to everything. Drainage for two hours a day, six inhalations for ten minutes - three hours already. During periods of exacerbation, even longer. I constantly monitor my vital signs - I measure my temperature and blood sugar level: I also got diabetes, so I inject insulin. I can't find a balance between hobbies, activism, friends, fun, sitting up to three in the morning on the Internet - ordinary teenage life - and the fact that you constantly have to control yourself. I cannot stay overnight with friends, because I have a strict regimen - I have to go home to do inhalations. Naturally, sometimes I violate it, but my health does not say thanks for it. You can skip just one week, and all the efforts a month before will go to hell. I always say that I am not the most exemplary patient. Not because I don’t want to live or I do not look after my health well - it just happens that way. It's really hard.
About the reaction of others
I switched to home schooling. Firstly, it is more comfortable to combine it with health care, and secondly, the Russian education system seems to me to be crooked. I don’t understand why it’s impossible to pass the tenth and eleventh grade programs in one year and just pass the exam. I don’t understand why spend two years on this.
But for nine years I went to school like everyone else. There were conflicts with teachers who did not want to let me out of the class: "Why do you even leave the classroom, what do you allow yourself?" But when you start to cough, you don't even have time to ask if you can get out - you just fly out of the office. Naturally, at first this caused misunderstanding, there were two marks for behavior. Then my mother was called to school, she explained what was what, and everything became fine.
I often run into prejudice. I recently read that a person with HIV was kicked out of an appointment with shouts: "You are re-infecting us all!" Can you imagine that a therapist does not know what HIV is, that it is not transmitted through a handshake? It's the same story with cystic fibrosis. People are afraid that it is something infectious, like tuberculosis. You start coughing and you have to get out of the subway car so people don't turn gray with fear. When a taxi driver looks at me, for example, because of a cough, I say: “Sorry, I have asthma,” and he understands that I am not contagious. Everyone knows what asthma is, and cystic fibrosis is something incomprehensible.
Or you come to a cafe. There are pills on the table, the waiter sees you eat a handful, and is immediately scared. Such a hostile look - you are perceived as a stranger, and this is always annoying. But it happens so often that I stopped paying attention. I also cannot explain constantly. If a person does not understand the first or second time, you simply stop contacting him. There are so many sidelong glances that if you look at each one, your neck will break.
About blogging and burnout
I started an Instagram account five years ago - I just posted ordinary photos from my life there. At some point, I began to read the news, take an interest in the fempovestka. My blog is not only about the disease - it has only become a reference book in recent months. Before that, I wrote about acute social issues: about my political views, feminism, news that cannot be ignored.At some point, I became interested in science and took up popular science education.
Once I said that there is corruption in the Ministry of Health, that the state positions itself as social, but does not fulfill its functions - it collects taxes, but ignores people with disabilities, pensioners, orphans. For some reason, it attracted a lot of attention - I did not count on this. Now my illness is the central topic of my blog. But this is temporary until the problem is resolved. I talk a lot about the situation with medications, talk to journalists, write. Sometimes I can't get out of bed because I feel sad and bad - I feel burnout. But I understand that I am doing a good deed - so why not continue?
When, for example, a taxi driver looks askance at me because of a cough, I say: “Sorry, I have asthma". Everyone knows what asthma is
and cystic fibrosis is something incomprehensible
It is impossible to please everyone, to find people with whom you will ideally coincide in views, therefore, naturally, conflicts arise. With activism in Russia it is infinitely difficult, many do not understand why a civil society is needed. It's hard to argue in the comments with people who don't want to be guided by common sense, empathy, and basic knowledge. Some people say, “I don’t want my disability treated for my taxes.” In the comments they write: "Parents should have thought!" The person believes that my parents, and apparently the parents of children with cystic fibrosis, are generally unaware of genetic screening. I realized that I need to tell what he is, that this is not a guarantee that the child will not have a disease, that not everyone has the money for it.
I easily abstract from other people's stupidity, but when there are a lot of people, it is, of course, more difficult to do this. Plus, communication is, in principle, exhausting. I have a lot of messages in my directory - both insults and praise. Recoil helps to cope, the fact that the people you focus on notice you. It also helps to relax - turn off the phone, go to the gym, take a walk, turn on the TV series, chat with friends and relatives. In general, blogging is difficult - but fun for me.
On the situation with medicines in Russia
Cystic fibrosis is incurable at the genetic level. But what the body cannot do on its own can be compensated for with drugs. For example, the absence of an enzyme can be compensated for with a pill with it, bacteria can be killed with antibiotics. The disease is multi-organ, medications are expensive, and you need a lot of them - and if you remove the drug or replace it with a low-quality one, everything in the body crumbles like dominoes.
For example, a vibrating vest - a special apparatus that helps to clear your throat - is very expensive, and a lot of money is needed for examinations. I think the state is not interested in spending money on this, it is trying to save money. Federal Law No. 44-FZ (Federal Law No. 44-FZ "On the contractual system in the procurement of goods, works, services to meet state and municipal needs" regulates, among other things, the procurement of drugs. Its priority is to support domestic manufacturers and save money. At the end of last year, experts began to note that foreign companies were withdrawing their drugs from the Russian market.”Many patients with cystic fibrosis noted that the necessary antibiotics had disappeared from the free market. Approx. ed.) - according to it, preference is given to Russian rather than European drugs. A person without cystic fibrosis, who has bronchitis or sinusitis, a Russian antibiotic will help. But for people with orphan diseases, people in serious condition, people with cancer, such drugs can be dangerous: they are not effective enough, they have unpredictable side effects. When a person weighs forty kilograms with a height of one meter and seventy (quite standard parameters of a person with cystic fibrosis in Russia), he simply does not need three days of diarrhea as a side effect - he will weigh thirty-nine kilograms, this cannot be allowed.
The legislation states that if an analogue drug does not suit you, you can tell the doctor about it and you will be prescribed an original drug. In reality, when you get a generic drug and you start having side effects, you still have to prove that you are not faking. Doctors do not want to fix it - they will rest on what you come up with. After the doctor finally agrees with you, you write a complaint to Roszdravnadzor - they are checking. After that, a medical commission is assembled, which decides whether it is necessary to give the original drug. When the drug is prescribed, a special purchase paper is sent to the Ministry of Health. The Ministry of Health can import this drug for a month, two, three, six months - and it is needed right now. So you have a choice: either you die without this drug - for example, in a week (with an exacerbation it is quite possible), or you have side effects - diarrhea and vomiting - and you die from them, only a little more slowly. Or you need to invent something and dodge - go to charitable foundations, break the law, collect money. By law, you can get yourself an original drug. But in reality, my parents and I have been trying to get it for the fifth month, since October.
About flash mob
In November, we raised the first wave of attention to the problem - we told what cystic fibrosis is, that lawlessness is taking place in the country. Journalists wrote about us, round tables were held, but on the New Year everything came to naught - in 2020 we decided to start again, with a flash mob.
Two months have passed since the beginning of the year - during this time, ten people have died. The essence of the flash mob is that the state strangles people - as if putting a bag on their heads. This is not only a method of torture - the system has a package for everyone: today you are healthy, but not tomorrow. There is also a literal meaning: because of oxygen starvation, people with cystic fibrosis can hardly breathe, as if they are in a plastic bag. The hashtag #LetMe Breathe is a story that, firstly, you need to give people medicine so that they can breathe, as well as stop choking those who, due to their physical capabilities, cannot answer.
I would like to reach out to independent YouTube journalists, media, public figures. On March 4, an agreed mass picket will take place (a group of parents of children with cystic fibrosis is holding an agreed action in the Moscow Sokolniki Park. Approx. ed.). I would like to solve the problem at the federal level - ideally, remove orphan diseases from the operation of Federal Law No. 44-FZ. As for me as a person, I would like to live until 2024 and see another president. I want to do journalism, activism - it's interesting to me.
COVER: Taisiya Sheremet / Vkontakte