Parents of children with cystic fibrosis and adult patients are concerned about the disappearance of the drug Pulmozyme from the Russian market.
Cystic fibrosis is a serious genetic disease in which the function of the excretory glands is impaired.
In Russia, according to the Ministry of Health, about 4 thousand people have been diagnosed with such a diagnosis, three quarters of them are children: patients rarely live to be 25 years old.
Patients with cystic fibrosis must constantly take a variety of medications, including those with antibacterial and mucolytic effects.
For more than 20 years, patients say, many have used the drug "Pulmozyme" (international non-proprietary name - dornase alfa) produced by the Swiss company Roche for daily inhalation.
In Russia, the medicine was supplied to the Pharmstandard - Ufa Vitamin Plant, where secondary packaging was carried out, quality control and further sales were carried out throughout the country.
However, in early March 2021, the Swiss manufacturer notified the Russian Ministry of Health and the Ministry of Industry and Trade that it intends to stop importing Pulmozyme into Russia from next February.
As the Pharmstandard - Ufa Vitamin Plant clarified, "the production of the drug Pulmozyme has been discontinued for an indefinite period."
“The decision to stop supplies was made taking into account the availability on the Russian market of a drug with the same international nonproprietary name in the same dosage form and with an identical route of administration,” says a letter from Roche-Moscow JSC (available to RT).
We are talking about the drug "Tigerase" produced by the Russian company "Generium".
According to media reports, Generium was founded in 2011 by Viktor Kharitonin, who also owns Pharmstandard.
Although the supply of Pulmozyme is due to end only next year, patients say that the analogue (Tigerase) was imposed on them a year ago.
Despite the fact that, according to the government procurement website, the regional health ministries continue to place applications for the purchase of Pulmozyme, it is impossible for private consumers to obtain it in sufficient quantities in pharmacies even for their own funds, patients say.
Cannot be replaced
A resident of the Kemerovo region, Natalya Rudenko, the mother of five-year-old Andrei, recalls that the diagnosis of cystic fibrosis was made when her son was only a month old: “He was urgently hospitalized, he was between life and death, he was on mechanical ventilation. At that moment, a four-year-old boy with cystic fibrosis was taken to intensive care. He died, after him there was "Pulmozyme", and it so happened that my son was inhaled with him several times and saw a positive trend. We did some tests and it turned out that Andrei also had cystic fibrosis. My son was on the mend, we were transferred to the ward, and the manager called me and said that such children live up to three years. "
According to Natalia, they continued to be given Pulmozyme until November 2020, after which the drug was replaced with Tigeraza: “We decided to try this drug because it suits someone.
But after four days of use, the child became lethargic, his oxygen saturation dropped, and shortness of breath began.
Submitted a notification to Roszdravnadzor about unwanted side effects. "
After that, the family was still not given Pulmozyme, even after Roszdravnadzor checked and decided to provide the child with this particular drug.
Moreover, Natalya Rudenko continues, local officials began to accuse her of being a swindler who receives Tigeraza and sells it, and representatives of the clinic tried to get to her home to see for themselves what drugs and how much are stored there.
“Later, the polyclinic conducted a medical commission, in the conclusion of which it was stated that Andrei's condition was stable, I fantasized the side effects, and what exactly he was breathing, they could not say, since I did not let them go home.
This document crossed out everything, because now we are required to go through this hell again with side effects from "Tigeraza" in order to fix them again.
Basically, I'm being asked to do a cruel experiment on my own child, ”she complains.
© Photo from personal archive
Tatyana Zavgorodnyaya, a resident of the Krasnodar Territory, also talks about the undesirable consequences of taking "Tigeraza".
Her son Dmitry is 12 years old, he has been diagnosed and disabled since he was three months old, and he has been taking Pulmozyme since seven.
For the first time, the boy inhaled Tigerase in July 2020.
“He immediately said that some medicine was strange,” his mother recalls. - The throat has a tickle, dizziness, nausea, discomfort, pressure jumped. I thought that it should be so, but on the fourth day the pediatrician said that it was urgent to return Pulmozyme. I handled side effects with problems, all last August the drug was not given out, I had to complain to the prosecutor's office. The local regional pulmonologist told me that if I want to receive at least Tigerase, I must refuse Pulmozyme. "
Svetlana Petrova from Sochi, mother of seven-year-old Ksenia, also faced persuasion from doctors to switch to Tigeraza on the verge of threats. “The child is very allergenic, so the conclusion of the medical commission says that it is Pulmozyme that is shown to her,” she says. “Last year, we were left without this drug for four months, my daughter stopped sputum, she started wheezing, shortness of breath and eventually ended up in a serious condition with bilateral pneumonia in the hospital.”
After numerous complaints, Svetlana and Ksyusha were again provided with medicine, but only until this summer: “When it became known about the termination of supplies to Russia, the chief pulmonologist of the Krasnodar Territory began to demand that I urgently abandon Pulmozyme, otherwise he would allegedly not be able to order even "Tigerase". This demand is ridiculous, stupid and sad. Every month for seven years I write, I call, I get it, and they give me not a medicine, but another stupid answer for 30 days, and everything starts all over again. "
Svetlana says that during the interruptions she had to buy Pulmozyme herself.
“We spend 60 thousand a month on it, but I live in a rented apartment, I raise a child alone, and for me this is a lot of money,” she says.
- Foundations do not buy this particular drug, because the state should provide them.
It is especially painful that in all developed countries, cystic fibrosis is no longer a serious diagnosis: people live normal lives, taking targeted therapy.
And we are being deprived of what slowed down irreversible processes. "
"Even the remnants have disappeared"
Muscovite Marina Rybalkina, mother of nine-year-old Ivan, also says that from the moment when polyclinics began to provide only Tigeraza, they had to buy Pulmozyme on their own: “It took 40 thousand rubles a month. But now even the remnants have disappeared. We have a supply until the end of summer, it is not known what to do next, without this drug, the lungs of patients will be inflamed and filled with sputum. There are ideas to do inhalations every other day, but will officials or manufacturers of "Tigerase" be able to breathe every other day? Hardly".
According to Elena, a resident of the Kaliningrad region, mother of two-year-old Lev Vasilievs, the difficulty in switching children to an unfamiliar drug is that little patients cannot always tell themselves if they feel worse. “The son has a delay in speech development, although otherwise he is a strong, developed, very quick-witted child. But if absolutely critical consequences do not arise, children will not be able to say that something is wrong, moreover, they are not able to compare their feelings after one and the second drug, ”she notes.
Elena has been seeking supplies of Pulmozyme since 2020: “In March, the pharmacy wanted to give us Tigeraza, we didn’t agree, we had to call the police, and only after checking we were given the last 15 packages of our drug.
Then we bought it ourselves all over the country.
At the end of 2020, "Pulmozyme" ended even in Moscow pharmacies, it was necessary to bring it from Poland, and this with closed borders. "
At the end of 2020, the prosecutor went to court to protect the interests of Lev Vasiliev, the court of first instance ruled that the child should be provided with Pulmozyme, but the regional Ministry of Health appealed and won.
Now Lev's parents are filing an appeal.
In addition, another trial is being prepared in connection with the change in the remaining drugs from the so-called basic therapy: earlier they were purchased by trade name, and now by international non-patented (INN), which allows replacing original drugs with generics.
"The daughter wants and can live"
However, not everyone who has lost a vital medicine has enough energy and time for legal battles.
For example, a resident of Tatarstan Yevgenia, mother of 17-year-old Anastasia, went to single pickets.
The girl was diagnosed at a year and three months, a disability was issued at a year and a half, since then she has been taking Pulmozyme.
“In February 2020, we changed it to Tigerase, but four months later it became clear that she was intolerant to this drug,” says Evgenia. - In the summer, the medical commission decided to cancel "Tigeraza", formalized the side effects, sent a notification about this to Roszdravnadzor. From that moment on, Anastasia was left without the drug. Against this background, her health began to deteriorate; in October 2020, it was even talked about emergency hospitalization. We were never given Pulmozyme, referring to the fact that the suppliers did not have the medicine, so we had to look for it through friends. In December, I went to a picket, the medicine appeared on the second day of the picket. But in March 2021, the interruptions began again, so I went out into the street with a poster again. "
Evgenia is unable to work, she is caring for a disabled child and receives 10 thousand rubles a month.
Anastasia is the only child in the family, according to Evgenia, the girl's dad left her as soon as he found out about the diagnosis.
“Her dad was just scared of the difficulties, I do not blame or condemn, not everyone is given the strength to fight the system for the life of their child,” the woman says.
“I love and respect my daughter, Anastasia is my pride,” continues Evgenia.
- She moved to the 11th grade, in the future she plans to go to university for a psychiatrist, she also wants to get a second higher education, to become a lawyer specializing in criminal law.
She is fond of drawing, writes poetry, books, independently began to study German.
She is a good, cheerful, talented teenager who wants and can live, but it just turns out that she can, if allowed. "
Disruptions in supplies are not limited to children: adult patients, due to the fact that the disease lasts longer, are more vulnerable both to possible complications from a new drug, and to the unwanted effects of changing the drug itself.
Tatiana, 30, has been taking Pulmozyme for 19 years.
“I have a difficult course of the disease, for several years now the question of a probable transplant has been raised.
From Tigeraza I had a wild side effect with bleeding, high fever and bronchospasm, ”she says.
According to Tatyana, in Russia it is still accepted to consider cystic fibrosis as a childhood disease: “Yes, people over 18 years old did not survive before, but now we are, and we have three times more problems.
We need drugs just like children, but getting them is much more difficult. ”
At the time of publication of the material, the Ministry of Health of the Russian Federation did not respond to an official request from RT.