Who needs you there: stories of families with children and adults with cystic fibrosis who have left Russia. They received treatment 'abroad' that they could not get in their own country

We planned to talk about the heroes of this material in a special issue dedicated to the most common genetic disease in the world - cystic fibrosis.

We wanted to speak about how people with this diagnosis live in Russia fight the healthcare system for medicines and their right to life. That release was scheduled for early March 2022.

By July, all our heroes had left Russia. At the same time, Moscow's Hospital No. 57, the nation's premier center for treating the few adults with cystic fibrosis who lived to be 18, fired one of the two leading pulmonologists specializing in the disease. Elena Amelina lost the opportunity to help patients from all over Russia, because she wanted to treat them too much: she prescribed expensive drugs at the state expense and helped patients through the courts to seek the main drug that saves their lives - the Trikafta genetic corrector.

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Cystic fibrosis is a hereditary disease that leads to the accumulation of thick mucus in the lungs and pancreas, disruption of other organs. In the absence of constant treatment, daily procedures and physiotherapy, the lungs fill with sputum, pathogenic bacteria and fungi start up in them.

Patients constantly experience exacerbations: high temperature leads to the formation of even more sputum.

Without expensive and high-quality intravenous and inhaled antibiotics, the body cannot defeat the infection. Clogged lungs stop absorbing oxygen. The person suffocates and dies of suffocation.

The average life expectancy of patients with cystic fibrosis in Russia is 12 years, in Europe and North America it is more than 50 years.

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Novaya Gazeta publishes three stories about Russians with cystic fibrosis who went abroad in 2022 and received treatment there that they had difficulty getting or could not get at all in their own country.

A matter of forty minutes

The Morozov family has two children with cystic fibrosis: Ulyana is 8 years old, Miroslav is 10. Due to inaccurate genetic analysis, Miroslav was diagnosed late - when he was almost three years old. Until that moment, the doctors had been convincing the parents, Pasha and Leyla, that they had “just such a sickly child” - “he doesn’t eat well, he gains weight poorly, and often catches colds.” Miroslav's condition was rapidly deteriorating, and only Ulyana's genetic test saved him - both were diagnosed in 2014.

Ulyana and Miroslav at Uniklinik
Photo from the family archive

For nine years, Leyla and Pasha have been fighting for the right to see their children as adults. They fight not only with the disease, but also with the state machine.

Since 2015, Leila has been blogging on Instagram about her family's life, creating educational content for other patients, helping to raise money for them and find medicines. To get the necessary drugs - original antibiotics with proven efficacy and daily therapy - you need to go through many instances. And often they still buy drugs with their own money, because government purchases can take months, and the disease cannot wait. Even the purchase of original drugs can simply be refused, because they are expensive, and the budget is “not rubber”.

For the most important medicine for her children - a genetic corrector that can stop the progress of the disease, Leila fought for more than a year and a half.

In 2019, together with the families of other patients, she launched the “#Give the dark breath” information campaign demanding that children and adults be provided with timely and high-quality treatment. The patients died one after another, the Ministry of Health was silent.

Miroslav, Ulyana and Baron at Pulkovo airport
Photo from the family archive

“We are accustomed to the idea that our children have a shorter lifespan and a more difficult path than others. But we fought. For 4,000 lives of emveshki (patients with cystic fibrosis. - ed.) in Russia, - says Leila.

It was never simple, but since 2019 it has become unbearable and hopeless. Most of the original antibiotics were removed from the Russian market due to the law on import substitution. Instead, they began to buy cheap and dangerous analogues from India, China and Dubna. The patients left one by one.

We fought for the purchase of targeted therapy for children: we went through endless medical commissions, approvals, obtaining certificates, altering documents, obtaining new certificates, through unnecessary and dangerous hospitalizations, on which it was possible to catch hospital infections. Finally, in June 2021, our family received confirmation of the annual purchase of a genetic corrector for Miroslav and Ulyana and the first packs of drugs in hand. It was happiness, but we knew that it was very fragile and unreliable. And yet our children got a chance.

In February 2022, we couldn't believe what was happening. We didn't think about leaving. I remember this time very badly, everything is in a fog.

In March, we realized that we were powerless to change something and that we had to go. Moreover, threats began to come from readers of my blog and even from other parents from the MV community for our position.

We were afraid of mobilization and that Pasha would be taken away, that the children would say something at school, and guardianship would come. We bought a cage for the flight of a dog and began to collect documents.

My husband is a programmer, his company offered to help employees with relocation. We had a direction - Germany, but there were no deadlines and no understanding of the future. The intermediate point was Armenia, where they had to deal with the legalization of the move.

After Leila announced her intention to leave on her blog, the persecution began. The commentators wished her and her children dead, wanted to be left without medicines, accused her of “ungratefulness”, because the state “gives them drugs worth several tens of millions of rubles.”

And most often in the comments there was a phrase: “Who needs you there?”.

In May 2022, problems began with the delivery of genetic correctors to Russia. Due to the sanctions, logistics became more complicated several times, purchases began to be delayed. The Krug Dobra Foundation, which provides drugs to children with orphan diseases, was forced to announce that it was impossible to fulfill many obligations on time and provide children with life-saving drugs on time. Parents began to divide the dosages of drugs for their children into halves and quarters in order to make it to the next purchase, and buy drugs from the hands of foreign patients.

Leyla and Pasha booked tickets to Armenia for the middle of July. Just before their departure, the Trikafta party nevertheless reached Russia. They took a minimum of clothes with them: out of three suitcases, two and a half were occupied with medicines for children, inhalers, breathing simulators.

They ended up in Frankfurt on Christmas Day. The children were immediately able to go to a German school, they were given tablets with an online translator, and they were assigned to additional language classes.

The first thing the teacher asked Layla with the help of Google translator was: “What can we do to make your children feel better at school?”.

Leila talks about the most important thing for the family of a chronically ill person - access to medicines - as if she does not believe herself:

— We received an appointment at the MV Center in Heidelberg on March 9th. "Trikafta" was the last pack. Pasha and I could only think about what we would do if the medicines were not given. One evening, he asked: “Do you think they might not give us a residence permit because we ask for such expensive drugs for children?” It is hard to explain how you can simultaneously feel horror and sticky cold and yell at your husband that he is an idiot.

That night I didn't sleep. On March 9th, we went to the first appointment at Uniclinic. It took the doctors 40 minutes and two documents to write out all the necessary basic therapy and Trikafta to the children - our St. Petersburg extract, translated into German, and a certificate from the oculist that due to possible side effects of Trikafta, the children did not develop cataracts. We received prescriptions on Thursday, and on Monday we were given all the medicines.

Ulyana and medicines issued in Germany
Photo from the family archive

Leila did not fully believe that this could be so. Even when I saw the prescriptions with my own eyes, I was afraid that some kind of mistake would come to light - after all, “they don’t even have a residence permit, who are they to treat their children here.” I didn’t believe it when I went to the pharmacy in the morning. And when she brought the medicine home, she wept.

The repeated purchase of Trikafta in Russia, despite the fact that the decision of the medical commission on Uliana and Miroslav was really a whole year, was never carried out. On March 13th, Leila informed the Krug Dobra Foundation that the children were receiving all the necessary treatment in Germany and that Russian purchases were no longer needed.

A right that cannot be taken away

Irina Dmitrieva, the former president of the Russian organization of patients with cystic fibrosis, was supposed to be one of the experts in the special issue of Novaya Gazeta. Now she and her daughter Marfa live in Bulgaria.

Marfa is 14 years old and was born with severe symptoms of cystic fibrosis.

All her life, Irina has been fighting for her daughter: with a disease, with Pseudomonas aeruginosa, eating Martha's lungs, with the Moscow Health Department, the Ministry of Health, the bureaucracy, the indifference of officials, medical commissions, and the lack of medicines.

Fighting for her daughter, she began to help other patients - to raise money for special food, antibiotics, inhalers, and trips to hospital in Moscow. And then she became the president of the patient organization.

Ira took upon herself the interaction with the state: endless meetings of the Public Chamber, round tables, letters to the ombudsmen and all circles of bureaucratic hell in order to at least slightly facilitate the access of patients to treatment, to life.

Irina Dmitrieva with her daughter Marfa
Photo from the family archive

In 2021, through the Circle of Good Foundation, Marfa received the coveted Trikafta. On March 7th, 2022, Irina flew to Bulgaria with Martha and one suitcase for two.

The decision was desperate. We chose Bulgaria because we have friends in Tsarevo. And one of my former students lives in Sofia. She arranged our first video call with a pulmonologist. And yes, it helped a lot.

On the way, somewhere between the check-in desk, the personal inspection of the suitcase by customs officers and the loading of the suitcase on board at Vnukovo, the most valuable thing that Ira and Marfa had was stolen from it - two packs of Trikafta.

At a transplant in Istanbul, Ira received a suitcase, which lacked medicines for several million rubles and two months of her daughter's life.

The appeal to the police did not lead to anything, the surveillance cameras did not show the theft or did not work. For several weeks, Marfa was without medication - until Novaya Gazeta correspondents brought them. For four months, Marfa drank half the dosage.

Then the Moscow Morozov hospital refused to issue documents for a new purchase without the patient's personal presence (although the decision of the medical commission was valid for a year).

Irina says:

– There are only 220 patients with cystic fibrosis in the whole of Bulgaria. With basic therapy, things are not very good here - the state buys only a few drugs, and you have to choose between them, you can’t get everything. But this is no worse than in many regions of Russia. However, since August 2022, they have been giving the most important thing - Trikafta.

We went to Sofia for the first appointment with the doctor - she is amazing! She hugged Martha, admired, just a fountain of emotions. Immediately at the reception, they gave drugs to take home, the antibiotic colistin and the enzyme creon.

Irina applied for political asylum in Bulgaria, but in December a negative decision came from the migration service. There was not enough evidence of a threat of persecution. At the same time, the denial of asylum did not affect the supply of medicines.

– We get Trikafta, and it will be so, because the authorities, by law, cannot deprive a child of the right to life. And the law works here.

Following Irina's complaint, the administrative court annulled the decision of the Bulgarian Migration Service to refuse asylum. The Migration Service has filed an appeal with the Supreme Court, the hearing is yet to come. But Marfa continues to receive treatment, since by law the child must be provided with medicines, regardless of migration status. Irina says that she and her daughter are not going to return to Russia.

"No more fear"

Taisiya Sheremet, an intern at Novaya Gazeta, and I left Russia for Europe in June 2022 by car. They loaded my minibus up to the ceiling with medicines: antibiotics, inhalation drugs, pills, dropper systems, syringes, saline for intravenous therapy, took with them two oxygen concentrators, an auto-refrigerator with medicine ampoules, three dogs and a couple of bags of things. In six weeks, short hauls, so as not to provoke an exacerbation of her disease, a long and difficult road in the heat and stagnation of sputum, they reached Spain. Friends helped us find an inexpensive house to rent in a campsite on the seafront in Valencia.

Tasya's genetic mutations are so rare that Trikafta, the most modern genetic corrector for patients with cystic fibrosis, cannot help her. She is one of the unfortunate 10% for whom targeted therapy has not yet been invented.

But research continues, and our goal is to wait for the magic pill. In the meantime, fight an unequal battle with low lung function, infections and coughs.

By January, we were able to sort out the documents required to obtain free health insurance in Spain. We don’t have a residence permit yet, but we have a residence permit in our friend’s house and a temporary medical insurance card. We were able to get it by explaining to the social worker at the local clinic the difficult circumstances of our emigration and stay in Spain. After half an hour of conversation, she handed us both small red cards with our data and said that she would help with the first appointment at the clinic, where we could write out everything we needed for the first time and a referral to a specialist in the MV center.

Two days later, the drugs were in the pharmacy near the house, and her owner Mercedes, who thinks I'm strange because I always come during the siesta, apologetically said that I would have to pay a little extra for the drugs. I said that I would pay for them in full, "only please give." I had to pay two euros and forty-five cents. The total cost of drugs was more than one hundred euros. Two out of five drugs have not been available in Moscow since the spring of 2022, two more since 2019.

In February, another exacerbation got out of control and I had to go to the hospital. Tasya's condition so frightened the doctor in the emergency department that the decision to send the patient to the intensive care unit was made immediately. Although in Russia we would be told that this is not necessary.

– They don’t force you to undress in public, they don’t take away your panties and your phone. The room is separate for one, no curtains. All staff change disposable gowns and gloves when they enter the ward,” says Taisiya. “Immediately they took all the tests and connected an intravenous antibiotic. When I saw what was written on the bottle with a dropper, I did not believe it.

When I saw the original tazacin on Tasya’s photo from the intensive care unit, I began to cry. There has been no original tazacin in Russia for more than five years.

When the results of the tests came in, the doctors transferred Tasya to the regular department and connected two more intravenous antibiotics - bramitob and linezolid. For such a course of intravenous therapy in Russia, the Oxygen Charitable Foundation has been raising funds for months. And often he cannot close the fees and pay off the pharmacies that sell the drug on credit by agreement.

Therapy in Russia is started not when the patient needs it according to indications, but when money is collected and the drug is available in the pharmacy.

Any visitors to whom the patient has given a pass with a barcode are allowed into the Spanish intensive care unit and regular departments. You do not need to be a relative and prove your right to see a loved one. True, only two passes are given per patient - Tasya's attending physician explains, apologetically:

- If you do not limit the number of visitors, then the whole large Spanish family will crowd into the patient's room, a terrible noise begins, this disturbs other patients and paralyzes the work of the medical staff.

In addition to antibiotics, the hospital provides all daily therapy - inhalation drugs, enzymes, vitamins, hepatoprotectors. They even bring sterile new inhaler cups.

In response to our explanations why Tasya is in such a state, the pulmonologist first frowns for a long time, then smiles softly and says:

“Don’t worry, we don’t have any problems with drugs, you won’t have to wait and achieve anything anymore. We will write out everything you need for every day at the time of discharge, you can get it at ten in the morning the next day at any pharmacy. If you need something urgently, there is a doctor’s phone and e-mail, call, she writes a prescription in the system, you have the medicine the next day.

During the three weeks of Tasya's hospitalization and before it, when I was doing the necessary paperwork, I interacted with several dozen employees of the La Fe hospital. I was never yelled at despite my poor and confused Spanish using an interpreter.

On the first day, when I, loaded with bags, was looking for how to get to her department, and accidentally ended up on the opposite side of a huge hospital, the guard gave me a mask because I forgot my own in the car:

“In general, you need to bypass this wing entirely, the entrance for visitors is on the other side. But your bags are so heavy... It's a holiday today, nobody's here, so come right through here. Just don't tell anyone, he said. He winked and helped me carry the bags. And I went to the elevator and again cried like a fool. When I told Tasya about this, she replied:

 I always had the feeling - that here I am, so small and weak. And there is a big and terrible cystic fibrosis, and there is also a big and evil state, and together with cystic fibrosis they are friends against me.

And this is so bad, hopeless and scary, and most importantly, powerless. What am I against them? And now for the first time in twenty years I have the feeling that I exist, my job is to do procedures, inhalation, drink vitamins, make droppers. And where to get them, how to defeat a bacterium, where to get antibiotics of a new generation, how to deliver them to me, this is the business of a big strong state and doctors who, together with me, are fighting cystic fibrosis. And from this it is so ... Not hopeless. And it's not scary anymore.

Source: Novaya Gazeta

Cover foto: Tasya Sheremet at La Fe Hospital, Valencia. Photo from personal archive