I'm Not Supposed to Die
// There is medicine to save the life of a boy
Pavlik Mitin is seven. He has Hunter's syndrome, a genetic disease that prevents the removal of some toxins from the body. In effect, the patient poisons himself. Children with the syndrome seem fine for their first two years. Then gradual deformation of the bones occurs, as well as deafness and inflammation of the internal organs. The child stops speaking, loses the power of concentration and the ability to walk. And then dies. Died, that is. Now a medicine has been developed.
I am sitting at the kitchen table with Pavlik and he is pulling on my hand with his own undersized and distorted hand, which is rough as a dog's paw. He seems to want to show me the family photo album. Pavlik is there, when he was two and still a happy tyke laughing, splashing in a poll and playing.
Snezhana, Pavlik's mother, says that when he wakes up, at 6:00, and his head is still clear, Pavlik can still talk. Now it is before lunchtime, but all I can understand “you” and “Pavlik” in the steady stream of noises he makes. Pavlik is tired.
They think that Pavlik no longer understands human speech. But his mother tells me about him as if he knew what she was saying. She says that, a year ago, when Pavlik could still talk, at his latest doctor's appointment, he suddenly refused to speak. The doctor wrote in his file that he had lost the ability to speak. After they left her office, Snezhana asked the boy what he wouldn't speak to the doctor.
“Lady pink boy tupid depf,” he answered.
His swollen tongue interfered with his speech. But that didn't mean that he liked it when grownups thought he was deaf or not smart.
|
|
i
For those who are encountering the Russian Aid Fund for the first time
The Russian Aid Fund was founded in 1996 to assistant the authors of desperate letters sent to Kommersant. We verify the letters with the help of local authorities, then publish the letters in Kommersant, Domovoi magazine and on the site www.rusfond.ru. If you decide to help, you will receive the banking details of the authors of the letters, and the rest is up to you. You just help you help. This approach has been popular with our readers. More than $8.4 million has been collected. We also organize relief efforts during national catastrophes, for 53 families of the miners who died in the Zyryanovskaya Mine in Kuzbass, 57 families of the policemen who burned to death in Samara, 153 families of the victims of explosions in Moscow and Volgodonsk, 118 families of the sailors who died on the submarine Kursk, 52 families of the hostages who died in the seizure of the performance of Nord Ost, 39 families of those who died in the Moscow Metro on February 6, 2004, 100 families who suffered losses in Beslan. The Fund is the winner of the Silver Archer award.
The Russian Aid Fund
Address: P.O. Box 50, 125252 Moscow, Russia
www.rusfond.ru
e-mail: rfp@kommersant.ru
Telephone: +7 (095) 943-9135
Telephone/fax: +7 (095) 158-6904
|
|
|
If I were Snezhana, I don't know what else I would do besides cry. But she is the strongest person I have seen in my life. She smiles. Five years ago, when her son was diagnosed with Hunter's syndrome, Snezhana, a lawyer by profession, taught herself all there is to know about the disease. The most important thing she found out is that there is a wonder drug in use in America called Elaprase. If it is taken regularly, as a diabetic takes insulin, the child's bones straighten and the powers of speech and reason return. The child can grow and lead practically a normal life, instead of dying at eight. The only problem is that Elaprase is not registered in Russia, and the producer did not want to register it and go through all the red tape for Pavlik alone. The company answered Snezhana that they would be willing to register Elaprase in Russia if she found 20 children with Hunter's syndrome in Russia. Then it would be of scientific interest to them to treat the children.
Snezhana found 39 children with Hunter's syndrome in Russia. She founded a nonprofit organization of parents of children with the disease. She negotiated agreements with Moscow Health Committee and Russian Ministry of Health and Social Development that they would pay for the Elaprase, as they pay for diabetics' insulin, if the producer registered Elaprase in Russia. Under Russian law, a medicine can be registered after it has undergone clinical testing. If enough of the medicine to treat Pavlik for just two months was purchased, it would be consider a clinical trail. And it would save his life. He would live, and so would the other 38 children in Russia with Hunter's syndrome. If the case enters the bureaucracy, Elaprase will be approved sooner or later any way, but Pavlik will no longer be here to benefit from it.
Pavlik is getting worse and worse. He can't always walk straight and is beginning to crawl again. His straw-blond hair is turning coarse and gray.
Snezhana recently took Pavlik to see a well-known professor of genetics. Pavlik, as usual, was mindlessly playing with things and seemed unaware of what was happening around him. The professor, thinking that Pavlik would not understand anything, told Snezhana that she was doing a wonderful thing and, thanks to her, dozens of children with Hunter's syndrome would survive it to lead healthy lives in Russia. But not Pavlik. With the bureaucratic hurdles involved, Elaprase is unlikely to be approved in time to save the boy. When he said that, Pavlik looked at him.
“I'm not supposed to die!” he told him.
&
1,085,450 rubles are needed to save Pavlik Mitin
“Pavlik Mitin has been diagnosed with mucopolysacccharidosis II (Hunter's syndrome) and has been under our observation since 2004,” says Prof. Petr Novikov. He is the lead specialist in genetics for the Russian Ministry of Health and Social Development and also work at the Moscow Pediatric Research Institute. He assured us that, if there were Elaprase, “the Pediatric Institute would accept Pavlik for treatment.”
In the West, the American medicine has been in use for several years, with impressive results. Children used to die between the ages of 6 and 12 from impairments of the skeleton, joints, brain and internal organs. “Its effectiveness in small children is simply excellent,” Prof. Novikov says emphatically. “There is physiological recovery, the functioning of the organs is normalized, toxins are eliminated.” Developmental delays are compensated for. “Therefore, we hope that Pavlik will improve both physically and psychologically with treatment with the medicine,” Novikov says.
Pavlik is a Muscovite, and “Moscow is responsible for providing him with lifesaving medicine,” the professor says. There are five children with Hunter's syndrome in Moscow, and 39 in Russia.
According to the correspondence between Snezhana and Moscow and federal health authorities, the medicine can be registered in Russia as quickly as in two months. That is, if the producer provides all the necessary documents (and it has assured us that it will) and the drug undergoes clinical testing. That means that, if the boy was provided with the drug now, it would save his life and the doctors would have a clinical test. In other words, the cost of saving Pavlik is 1,638,000 rubles. That is the cost of Elaprase for eight weeks. It takes two vials of the medicine per week, and each vial costs ˆ2925.
As always, our permanent partners, Ingosstrakh insurance company and the Capital investment group, have contributed $11,500 and $10,000, respectively (the details can be found at ). Thus, another 1,085,450 rubles is lacking. Money can be sent to the producer (a minimum of ˆ2925) or transferred to the savings of Pavlik's mother, Snezhana Mitina, in rubles. Any contribution will be accepted with gratitude. The Fund has the necessary banking details.
The Expert Group, Russian Aid Fund
Valery Panyushkin for the Russian Aid Fund
All the Article in Russian as of May 18, 2007
|
