"The state is trying to kill us"

Four thousand patients with a genetic disease of cystic fibrosis are devoid of vital drugs due to the law on import substitution

The morning of 11-year-old Martha begins an hour and a half earlier than the usual fifth grader. She drinks medicine. Makes special charging "kinesitherapy" to help their lungs open up. Drinks another medicine that lures sputum in the lungs, mucolytic. He clears it with a special device. And then in pure lungs make inhalation with an antibiotic.

At this time, Dasha, she is 23 years old, but look no more than fourteen, stands in a single picket at the door of the Ministry of Health. She has balloons and poster in her hands: “I want to live!” On the sidewalk next to Dasha is an oxygen cylinder.

In the same morning, the famous blogger Tasya Sheremet writes a post to his 62 thousand subscribers. She tells them daily about the life of an 18-year-old person with a rare genetic disease.

Tasi, Martha, Dasha and four thousand people in Russia with cystic fibrosis. This is the most common genetic mutation on the planet. Every 12th person is a carrier of a defective gene. The probability of the birth of a sick child in two media is 25%.

As a result of a mutation of a genet that affects the permeability of cell walls for a certain protein, in patients, all the secrets of the body (mucus) are very viscous and thick. This disrupts the operation of the respiratory and digestive systems. The cystic fibrosis is unreasonable and does not affect human intelligence. With timely diagnosis and necessary treatment, patients live in a completely rich life.

In his blog this morning, Tasia Sheremet writes ( spelling and punctuation are preserved. - Ed. ): “I just want you to know - the state is once again trying to kill us. There are 4,000 to us all over Russia, each of us has cystic fibrosis. Cymencidosis without adequate treatment is a painful and very terrible death from infection, suffocation, thinness.

Food is not absorbed, constant diarrhea and vomiting. A person does not gain weight at all, and light mucus clogs the lungs, different bacteria that cause intoxication start in it. A person is in a fever. The mucus prevents you from breathing, you are drowning in it, the lungs stick together and grow together, then ruptures, bleeding and scars appear. Then the liver cirrhosis, the kidneys refuse, does not withstand the heart - tachycardia, shortness of breath, fainting, oxygen starvation. The psyche is also handing over: depressive episodes, unwillingness to be treated and suicidal thoughts ... Well, you know the end. In Russia, it occurs at 18-20 years.

Is it scary? Do you know what is worse? To know that with cystic fibrosis you can live differently, know that in the whole civilized world there are no longer these horrors.

Instead, free basic therapy with which MV patients live happily to retire. Great true? "

In the countries of North America and Europe, patients with cystic fibrosis give birth to healthy children and even nursing grandchildren, study, work, travel. To give birth to a healthy child, they only need to ask a partner to pass a genetic test and make sure that he is not a bearer of a defective gene. The average life expectancy of patients with cystic fibrosis in developed countries is 50 years. And it is constantly growing.

The fifth generation of targeted therapy preparations, which are fighting not with symptoms, but with the very cause of cystic fibrosis, has been published in the United States. These drugs make the desired protein mobile that ceases to get stuck at the cellular level. Thus, they reduce the viscosity and density of the secretions of the body and reduce the symptoms of the disease to a minimum. Targeted therapy has been used throughout the world for seven years. None of these drugs still have been registered in Russia, and its cost for ordinary families is prohibitive: for one patient, an annual rate of about $ 300,000 and these pills must be drunk for life.

Mom Martha, Irina, worked for 15 years as a teacher at school. And now she is the head of a public organization, which helps to establish systemic care for patients with cystic fibrosis in Russia. Her life was connected with this disease long before the birth of her daughter: “I had an episode. I saw on TV a video about the department of the RDKB, it was talked about cystic fibrosis. Then I only graduated from school, but for some reason he made such a deep impression on me that I stopped and watched to the end, although I walked past the TV. I still remember this boy, his mother, who gave an interview. I then heard this word for the first time - cystic fibrosis. I even remember my thoughts at that moment. So deeply I hit me then. But then I forgot about this plot, as it seemed to me, forever. And I gave birth to my daughter at 35. And when they called me after the screening and said that the big risk of cystic fibrosis, the outbreak occurred so bright - I instantly remembered this plot, although 17 years passed. And immediately I understood everything ... "

Martha goes to a regular school, and in his free time draws animation. She shows me a short video in which the man’s wings grow, and he flies out of the frame: “He flew away from problems,” Martha explains. While Irina and I are discussing why medicines for patients with cystic fibrosis disappear from Russia and listen to the performance of the head of another public organization on the radio, Martha conducts its evening ritual, which repeats the morning and also lasts an hour and a half, with a bickering and inhalations. Then she looks into the kitchen, comments on the words of the journalist on the radio:

“To increase life expectancy” - so they say that I will die soon. The wrong wording, I do not agree with her. ”

We have never had such miracles as the obligatory provision of portable oxygen cylinders or compressor vests that help get rid of sputum, for each patient. They can be seen at them in the recently exploding world rental film about patients with cystic fibrosis in the United States "a meter from each other." But still, the rise in the quality and life expectancy of patients fell for the period 2008–2013. Then in Russia (more precisely, most in Moscow) there were original enzymes and antibiotics in the public domain. The patient was given a prescription, he went to the pharmacy and immediately received the medicine. In one day. Outpatient observation of patients has become a priority. The risks of hospital and cross infection have decreased, the treatment was high -quality and safe.

In 2014, Federal Law No. 44 on the purchase of medicines entered into force (Clause 6, Part 1 of Article 33). It regulates state purchases of drugs, including for patients with cystic fibrosis. The priority is the support of domestic manufacturers, import substitution and saving of budget funds. Instead of proven and effective drugs, cheaper analogues began to be purchased, the so -called generics. Several manufacturers of cheap generics go to the state contract, and someone alone wins it according to the obvious criterion of cheapness. For several years, this mechanism forced foreign pharmaceutical companies to first reduce prices to unprofitable, and then completely leave the market. In November 2019, the last antibiotics vital for patients with cystic fibrosis disappeared from free sale.

The senior researcher at the Kygistzidosis Laboratory of the Federal State Budgetary Institution of the Pulmonology, Stanislav Krasovsky, into the department, which patients with cystic fibrosis of over 18 years old in the most serious condition from all over Russia, explains the current situation: “The problem is not in generics, but in the system itself. We, doctors in the hospital, work with what we are purchased, and we have a very conditional effect on the choice of these drugs. There everything is dictated by economic considerations. This month, one drug is cheaper, it was bought, and in the next there may be another drug, and then the third. When we were purchased high -quality, proven drugs, we treated according to the so -called gold standard. Even with such a dosage scheme, for example, antibiotics for patients with cystic fibrosis, maximum, and a course two, or even three times longer than ordinary patients. With such a treatment regimen, the quality of cleaning the drug, its toxicity, side effects are critical.

And generics are often needed by the dose several times more. We try, select, adjust the dosage. And in the next month, another drug is brought, because there are dozens of them. Russian, Chinese, Indian production. And the biggest problem with them is that they were not tested on patients with cystic fibrosis. That is, not only do not have proven clinical effectiveness with this diagnosis, but can also have side effects that threaten life, which we learn only when we begin to use the drug on a particular patient.

And in order to prove that the drug, for example, does not work, or that it has life -threatening with cystic fibrosis, side effects, the doctor should fill a ton of papers, forms, reports for each such case.

This is physically impossible. So I should have an eight -hour working day. Today I am leaving the hospital four hours later, twelve hours it turns out. And from all this time, interaction with patients itself is allotted for only an hour, for all the byputs, for everything. Hour. Everything else is endless commissions, meetings and waste paper! Reports, plans, forms, forms! Militable bureaucracy. Doctors are forced to spend time on her, which patients do not have. ”

What is happening contradicts even the perverted logic of the state machine. In Russia, fifteen operations are carried out annually for patients with cystic fibrosis for lung transplantation. This is a federal target program that is subsidized from the state budget. The cost of one such operation is more than 10 million rubles. For many patients, this is the only chance of life, they can stand in line for transplantation for years. But even if they are among the lucky ones who waited, then after the operation they need potent antibiotics and immunosoding drugs that will not allow the body to tease other people's lungs. Now all these drugs have been replaced with cheap analogues. It turns out that the state spends more than 150 million rubles a year on transplantation of light suicide bombers. In order to save then, releasing cheap medicines for them. The patient dies, and death is written off to the severe stage of the disease. Because it is almost impossible to prove that he died of poor -quality drugs, taking dozens of drugs at the same time and having a serious genetic disease.

Throughout Russia, single pickets continue, on which patients with cystic fibrosis, their relatives and even doctors come out. Protesters demand to return the medicine and comply with the right given to them by the Constitution of the Russian Federation - the right to life.

If, after a few weeks, antibiotics such as Merinem, Fortum, Tienam, Kolistin, as well as other proven drugs will not return to free sale in pharmacies and providing hospitals, then four thousand patients with cystic fibrosis will begin to die. But before they die, they will suffer from suffocation, intoxication, liver failure and other internal organs. And only after that they will die a painful death.

A petition in support of the right of patients with cystic fibrosis for treatment with drugs with proven clinical effectiveness and more than two hundred thousand people have already signed a quality life.

On November 29, at a round table in Roszdravnadzor, where representatives of the Ministry of Health and the Ministry of Industry and Trade of the Russian Federation were invited, the heads of non -profit organizations that help patients with cystic fibrosis, practicing doctors and patients themselves, discussed the disappearance of original vital drugs. Officials promised representatives of funds, doctors and patients to negotiate with producing drugs. At the moment, there are no changes in the situation. The next round table was scheduled for December 11, 2019.

How to help

It is possible to support the charity fund for patients with cystic fibrosis "oxygen" here.

Details:

Charity Fund "Heat Hearts"
TIN 7701359136
GPP 770101001
Recipient Bank: Branch No. 7701 VTB Bank (Public Joint -Stock Company) in Moscow
BIC: 044525745
K/s: 30101810345250000745
R/s: 40703810500000034685