Parents Battle Officials As Siberian Kids With Cystic Fibrosis Face Infection Outbreak

KEMEROVO, Russia -- For nearly two years, Natalya Drobysheva has been locked in a battle with local health authorities in this south-central Siberian city to obtain the medicine she believes could save the life of her son, 15-year-old Ilya.

"While the department was dragging out the matter, my son was slowly dying," Drobysheva told RFE/RL.

"[Health] department officials told the court that our son did not need this medicine," she said, about a December 2016 court hearing. "An official with the Kemerovo Oblast Health Department, who was not a doctor, told the court: 'What if the child dies from this treatment?' She made me out to be an irresponsible mother and even said they were giving my son everything necessary. It is true that they offered us another medicine," she said, but added that doctors in Moscow told her it wouldn't work for her son.

"I told all of this to the court," she added. "And I cried. Even the judge couldn't take it. She quickly ended the hearing and issued a ruling in my favor."

Eighteen months later, Ilya Drobyshev is still waiting for his medicine.

Drobyshev has cystic fibrosis -- a genetic disorder that affects the lungs and other organs, makes breathing difficult, and leaves patients extremely vulnerable to lung infections.

In 2016, he was additionally diagnosed with cepacia syndrome, an opportunistic infection caused by the bacteria Burkholderia cepacia that is difficult to treat and potentially fatal to patients with cystic fibrosis.

In all, 22 children with cystic fibrosis in Russia's Kuzbass region -- nearly half of all the registered cystic fibrosis patients in Kemerovo Oblast -- have been diagnosed with the infection.

All of them have the same strain of the bacteria, meaning it is nearly certain they infected one another.

Doctors at Moscow's Russian Children's Clinical Hospital (RDKB) have prescribed an inhalable antibiotic called Cayston, produced by the U.S. pharmaceutical company Gilead Sciences, for the children, among other drugs.

"They simply told us to buy the drug on our own," Drobysheva said. "And sent us home. I understood immediately that no one was just going to give us this drug and that I would have to go to court."

A year's supply of Cayston costs 3 million rubles ($48,500) and, according to Russian law, it should be provided free to patients with so-called orphan diseases, the name for conditions like cystic fibrosis that affect relatively small numbers of people. There are some 3,000 registered cystic fibrosis patients in Russia.

'These Children Might Die'

Last summer, Drobysheva wrote to the Kemerovo Oblast Health Department requesting the medication, but officials did not answer her until she filed a lawsuit in November.

"They told us this medicine is not registered in Russia and that they cannot order it," she said. "Instead, they suggested [an Italian-made antibiotic] Fluimucil, but that medicine only makes his internal bleeding worse."

On December 12, 2016, the regional court ordered the Health Department to provide Cayston to Ilya Drobyshev. In view of the life-threatening nature of his illness, the court also ordered officials to comply with the request immediately, even if they intended to appeal the court ruling.

"The department waited until the last minute to file its appeal and didn't pay any attention to the fact that the court's order had to be fulfilled immediately," lawyer Maria Lazutina, who represented the Drobyshevs, told RFE/RL. "We asked court bailiffs to enforce the decision. We initiated enforcement proceedings. But to this day, the court's order has not been fulfilled. And without this medicine, these children might die."

In response to questions from RFE/RL, the Kemerovo Oblast Health Department sent a written statement confirming that they submitted the request for Cayston to Moscow only on May 11, six months after the court's order. The statement cited "unavoidable circumstances and difficulties outside the department's zone of control" to explain the delay.

The statement further said that a contract for the purchase of Cayston was signed earlier this month and that the Drobyshevs should get the medication within 30 days, exactly one year after Natalya Drobysheva sent her first request for the drug to health officials.

'Can't Wait Forever'

It remains unclear whether the families of the other infected children will also be given Cayston.

"I was told that an order for the medicine had been submitted," Tatyana Nazarkina, who has two children diagnosed with cepacia syndrome and who has also been waiting for more than a year to get her Cayston prescription filled. "But I don't know when we might get it. 'Wait until we purchase it,' they said. But these children can't wait forever. They are much too sick."

In addition, many of the parents of the children with cepacia syndrome believe the infection may have been spread by the poor hygienic conditions at the Kemerovo city hospital. According to general practice, cystic fibrosis patients should be treated in isolation from other patients because of their elevated susceptibility to infection.

But the parents, who have been exchanging information on social-media forums, say the city hospital treated the patients in open wards.

The current outbreak of cepacia syndrome bears many similarities to a 2008 outbreak in which 67 children were infected. That outbreak was traced to the RDKB children's hospital in Moscow, where children from across the country are sent for regular treatments. The strain of the bacteria in those cases was the same as the one in the current Kuzbass outbreak.

"Where could the bacteria have come from?" Yelena Kondratieva, a physician and head of the cystic fibrosis department of the Russian Academy of Medical Sciences who traveled to Kemerovo in May to help local officials improve the quality of their hospital care, told RFE/RL. "Only from there, from RDKB. After treatment, one of the children brought the bacteria home and during a checkup at the local hospital, passed the infection to other children."

The Kemerovo hospital was remodeled after reports about the cepacia syndrome outbreak, with two isolation wards being installed, some sanitation facilities upgraded, and the entire building disinfected.

Networks Alarmed

An inspection by Russia's consumer-protection agency, Rospotrebnadzor, in July 2016, found no violations at the facility.

But in August 2017, the All-Russia Association of Cystic Fibrosis Patients and other health organizations issued a statement reminding hospitals that people with the disease should be treated in isolated facilities with separate bathrooms.

"In numerous studies it has been demonstrated that the life expectancy of a cystic fibrosis patient with [cepacia syndrome] is reduced by 10 years," the statement said.

Kemerovo health officials told RFE/RL that no link between the outbreak and the hospital has been established.

Nonetheless cystic-fibrosis support networks are alarmed.

On June 11, the Novosibirsk NGO The Altruism Gene posted an alert on its VKontakte social-networking site.

'Why Did You Give Birth To Me...?'

It warned that some of the patients diagnosed with cepacia syndrome in Kemerovo were being treated at a special clinic in Tomsk.

"The parents of our children [in Novosibirsk] planned to travel to Tomsk in the very near future for genetic testing," the statement said. "It is no secret for anyone how the situation concerning cross-infections is in Russian clinics. Even today, the grown-up children who were treated at RDKB during the [2008] outbreak of this dangerous infection are still dying. We must do everything to prevent a repetition of this tragedy."

Yelena Kondratieva, the head of the cystic fibrosis department at the Russian Academy of Medical Sciences who consulted in Kemerovo, told the daily Izvestia last year that this fear is well-founded.

"Unfortunately, our medical facilities [in Russia] are not adapted to the needs of this category of patients," she said. "This is a widespread problem. A situation like the one that we see now in Kemerovo could develop in other places as well. Our goal is to do everything to prevent this."

For now, Ilya Drobyshev's condition has been stabilized by a cocktail of drugs, but the longer the Burkholderia cepacia bacteria remains in his lungs, the bleaker his prospects become.

"The longer we go without Cayston, the worse Ilyusha becomes," his mother told RFE/RL.

"In order to survive this, we have appealed to foundations. I have been helped by the mothers of other sick children and I buy some medications with my own money. Ilyusha needs a lot. Before we can leave the apartment we have to assemble a whole backpack of pills, injections, and other things."

"Ilyusha has really been tormented by his disease," she added. "You know, once he broke down into hysterics. 'Why did you give birth to me like this?' he said. 'Why? I am sick and tired of chewing up these mountains of pills.' I tried to comfort him, saying: 'You know, some people get by without legs. Others have no eyes and they get by. You have to push yourself to live -- there is no other way.'"

Written by RFE/RL senior correspondent Robert Coalson based on reporting by Ivan Smirnov, a correspondent for the Siberia Desk of RFE/RL's Russian Service