Paulette Barrantes, 17, faces every day with patience, lying in bed, waiting. A random victim of a rare bacterial illness, she is lucky to be alive. Following a sudden fever, a rash immediately appeared on her chest, arms and legs, and swelled into purple, fluid-filled boils. Nearly two years later, in her room in her mother’s small and humble home in Guadalupe, in northeastern San José, Paulette recalls how the boils on her arms, and then later on her legs, burst and ran with blood and the skin slowly turned black, dying.
Now, scars stretch from her wrists to her shoulders – a textured map of the painful history. On her left hand, only her thumb remains intact, as her other four fingers have been amputated at the middle knuckle.
Under the blankets that she keeps pulled up to her stomach, her legs and feet are bandaged – no scarring there, despite how much she wishes there were. No scarring, no skin. Just bloody tissue, muscle and bone.
After the rash burst, the skin on her legs never healed. All 10 of her toes have been amputated, and both her Achilles tendons have been removed.Her heel bones protrude – dry – from the backs of both feet.
A Common Bacterium
Staphylococcal purpura fulminans, as the illness she is now recovering from is called, began appearing in the United States just a few years ago, leaving few living victims. The first paper on the disease was published in April 2005 in the journal Clinical Infectious Diseases, according to the paper’s lead author, Dr. Patrick Schlievert.
“For the first few years, everybody who got it died,” Dr. Schlievert, a professor of microbiology at the University of Minnesota, in the United States, told The Tico Times in a phone interview Oct. 12. “It wasn’t until we started developing other strategies that people started to survive.”
Dr. Schlievert explained the disease Paulette is suffering from comes from a common bacterium called Staphylococcal aureus.
“About 50% of us have it in the nose. But we don’t all have the same strain,” he said.
“When we get a respiratory virus or get really run down, it can get into the lungs, make its toxins and send them out into the blood stream. Then, they massively attack the immune system.”
In some people, the result is the more commonly known toxic shock syndrome, characterized by a high fever and a sudden drop in blood pressure. Dr. Schlievert, who says he was one of the researchers who first identified toxic shock syndrome, estimated that 35,000 people die from the syndrome every year in the United States following an onset of the flu.
While some people get toxic shock syndrome, others, according to Dr. Schlievert, get staphylococcal purpura fulminans because of a genetic predisposition. When the bacteria attack, they cause blood vessels to break down and leak into surrounding tissue, he explained. People who are predisposed to blood clotting problems experience severe clotting and leakage – which is what forms the purple, blood-filled rashes associated with purpura fulminans.
“Purpura means purple blood, and that is because the blood vessels have been destroyed,” Dr. Schlievert said. “Fulminans means it takes a highly aggressive, fulminating course.”
In Paulette’s specific case, Dr. Schlievert said it sounds like the bacteria was not fully removed from her wounds, and is keeping the skin from growing back.
“I would encourage her doctors to put her on IVIG (intravenous immune globulin).
I think she has a greater chance of healing faster if she is on that,” Dr. Schlievert said. “Sometimes physicians don’t like to give it because it’s $2,000 per administration. But some people will never heal if that is not given along with the treatment.”
The Tico Times inquired with the Costa Rican office for the pharmaceutical manufacturer Bayer – a manufacturer of IVIG, according to Dr. Schlievert – but was told the company does not have the drug here.
Paulette’s doctor was not available for comment on her treatment because he was out of town this week.
Paulette’s Story
Paulette became sick Dec. 27, 2004 – “a day like any other,” she says – with what seemed like a cold, or flu. Her mother, Jocelyn Villafranca, 35, took her to a nearby clinic in Guadalupe, where she says doctors put her under observation, baffled. After eight hours, a frustrated Villafranca whisked Paulette off to the CalderónGuardiaHospital in downtown San José. There, doctors diagnosed the disease, put Paulette into the Intensive Care Unit, attached her to machines that would keep her heart, lungs and kidneys functioning, and told Villafranca her daughter probably had 72 hours to live.
Paulette wasn’t told about the grim forecast, but “a nurse asked me if I was prepared to go be with God. That scared me,” she told The Tico Times.
Paulette beat those odds, and spent the next nine months in the hospital. While expensive patches given to her as a trial offer allowed the skin on Paulette’s arms to heal, close and scar, the patches ran out and the regular gauze used on Paulette’s legs did not do the same. Her wounds wouldn’t heal, and the skin wouldn’t grow back. Her doctor at the Calderón Guardia public hospital in downtown San José said he saw no other option but to amputate both Paulette’s legs.
Paulette and her mother refused, and at the public Hospital México, on the other side of the city, a reconstructive surgeon successfully grafted skin onto Paulette’s feet by sewing her feet to her buttocks. Paulette laid on her stomach for two months with her legs bent and her feet attached to her rear end before the skin was cut from her buttocks and her legs straightened again.
In September 2005, Paulette was finally taken home. The treatment ever since has consisted of regular checkups at the CalderónGuardiaHospital and home visits from a nurse– now three times a week – to replace the bandages wrapped around her legs. As of six months ago, Paulette is using the same patches that worked for her arms on her legs, thanks to a donation from the U.S.-based manufacturer Ferris and the Costa Rican distributor Tri DM.
Lying in her room, her reddish hair pulled back tight into a short braid, Paulette is surrounded by a mix of reminders of her past and present. On the wall she faces every day hangs a large photo taken at her 15th birthday party, several months before she got sick. She beams at the camera from a dance floor, glowing. Shoved under her computer desk are boxes of surgical gloves, syringes, and vitamins.
By the side of her bed are her school books,and she looks to test past the ninth grade this month, thanks to a study-at-home program through the Public Education Ministry.
Before getting sick, she dreamed of a career in hotel administration. Now, because of the experiences and people she has met during the past two years, she says she wants to be an anesthesiologist or a reconstructive surgeon.
The pale and serious teen says she has been able to deal with past two years thanks to patience and her belief in God, though she says she sometimes feel like he is watching her from very far away.
How to Help Personal Note:
As a reporter, I live to write the stories that can make a difference. This, I hope, is one of those.
Meeting Paulette, I was struck by her strength and a combination of acceptance and the will to fight. She refused to end the constant pain and suffering that keeps her in bed by allowing doctors to amputate her legs. She continues to study, and dream about the future.
“In the case that I don’t walk again, I wouldn’t be the first person or last person in a wheelchair,” Paulette told me.
Since Paulette got sick, her mother Jocelyn Villafranca, a single parent of two daughters, has fought for Paulette’s recovery. Their resources are few, and their allies scarce. They need help.
Zolangie Zúñiga, a distant relative to Villafranca, has taken up the cause the best she can since February 2005. Tomorrow, Zúñiga is holding a benefit fashion show, titled “Wishes of Venus,’’ and featuring the swimwear designs of Desiree Morffe, at the Doce Lunas hotel in Jacó, on the central Pacific coast.
Entrance costs ¢3,000 ($5.75) and the money raised will be used to buy a special $7,000 bed to help Paulette’s circulation and – doctors hope – help her legs to heal.
Paulette has been making jewelry and small shoulder bags to sell. If you are interested in helping Paulette, or buying some of her handicrafts, contact Zúñiga at 643-2510. The family has also set up a bank account at Banco de Costa Rica under the name of Jocelyn Villafranca, number 1416014.
