Alina is 14. She lives on Viagra. This often invites smiles and at times cheap jokes. But Alina is not amused. She knows that Sildenafil, a drug sold as Viagra and by other trade names, is a medication used to treat not only sexual dysfunction but also pulmonary arterial hypertension (PAH).

In the United States Sildenafil used for PAH is called Revatio, to avoid being confused with Viagra. Alina also knows that even with Viagra (Revatio) her heart and lungs should have already failed. She has structural heart defects in addition to PAH. Such patients usually do not live long without transplants. Somehow Alina is still alive.

“Did you really make it yourself?” I ask, while looking at a blue rag cat with a loony face.
“My teacher helped me.” Alina says as she coyly wrings her hands. When it’s cold outside, she can’t go to school. Even the slightest chill results in an immediate cold, if not pneumonia. Hence the girl ends up missing most school days. When they can, teachers make home visits in order to give her lessons.

I compliment her on the cat and her other handicrafts. Actually, the girl looks like a healthy teen. Just her fingernails — bluish, dome-shaped like a wristwatch glass — reveal her condition. Her peers do not treat her well. She is different, a pariah. She can’t ride a bicycle, can’t dive in a village lake, can’t run and jump, and she can’t even walk longer than one hour without gasping.
You can feel what she feels by closing a plastic bag around your head and trying to breathe for a while.

Congenital heart defects (and Alina has a few) increase blood pressure in the lung vasculature, narrowing the blood vessels and making most of them dysfunctional. This causes severe shortness of breath, and unfortunately it only gets worse.

Breathing_03When we applied for child custody, everybody said we were nuts. “You must be crazy,” they said, “she’ll die anyway and then you will be blamed for negligence,” says Natalia, Alina’s mother.
Her birthmother left the baby in the street. Another family wanted to adopt her but when they were told about Alina’s congenital pathology, they changed their mind and left the girl at the hospital. They simply never came back after one visit. Natalia, who was then nursing her younger son in intensive care, consoled the little girl.

She just couldn’t leave the hospital without Alina. “It took us a while to get used to one another,” says the father. He has palms black from work, and a soft kind voice. “She kept aloof and had an ‘orphanage syndrome’: the louder you shout, the more chance you’ll be heard. At home it’s not like that. And now Alina is family.”

Her new brothers adore her. The older one is at war in Donbas, so the younger one patronizes the little sister for both of them.

Alina is complaining to me how she can’t draw well and how it upsets her. Her eyes are tearing. Her brother notices and brings her tissues.

He is 16, and, unlike his classmates, has no smart-phone or even a computer. “That’s nothing,” he says. “My happiness is elsewhere.”

Without warning Alina’s cheeks suddenly turn black. Right in front of our eyes. It’s time to “connect”.
Meekly, the gЦirl inserts two transparent tubes in her nostrils and lies down on a couch. The tubes come from what looks like a huge “suitcase” but is in fact an oxygen concentrator. She should actually use the concentrator for breathing around the clock. But it’s too hefty to carry about, and its mobile version is beyond the family’s means. So Alina can only breathe deeply while at home.
Pulmonary arterial hypertension is a rare disease. In the world, only five out of one million people suffer from it. There are some 200 patients with PAH in Ukraine. The only true remedy is a lung transplant. But Alina also needs a new heart.

Breathing_04

Such a surgery is extremely complicated and expensive — approximately $500-600K. A clinic in Cleveland agreed to put Alina on a waiting list. Now the Ukrainian PAH Association is busy preparing the required documentation and waiting for the bill.

But time is running short for Alina.

Daily drugs just to keep her going cost about $3K every month. It’s getting increasingly hard for the family to find the needed funds. They have nothing of value left to sell, and have taken a few loans. They’ve been buying Viagra while they could. Now they can’t even afford its Ukrainian substitutes.
Slowly and steadily, Alina is breathing through the tubes, fiddling with another crafted cat.

“Maybe I could make more of these and sell them at an auction? This is what they do now, so I heard. Do you think anyone would care?”

I see hope in Alina’s eyes…

By Marichka Paplauskaite
Photo credit: Олександр Хоменко
Originally published in Ukrainian
at life.pravda.com.ua

You can help Alina directly by making donations through her mother’s bank account, or via Association of Patients with Pulmonary Hypertension (see information below) to assist with arranging her treatment in Cleveland.

Bank name: Privatbank
Beneficiary: Natalia Gordienko
Account number: 5168757291102525
SWIFT: PBANUA2X
Or
Bank name: Privatbank
Beneficiary: Ukrainian public organization “Association of Patients with Pulmonary Hypertension” (PHA Ukraine)
Account number: 26008053146135
SWIFT: PBANUA2X