February 6th, 2007


Mimi's Distress: A modern case of Porphyria

Light sensitivity keeps Alhambran, 18, isolated
By Cortney Fielding, Staff Writer
Article Launched: 02/03/2007 11:27:23 PM PST

ALHAMBRA - Almost every light is out in the Alvarez home, but the family's 18-year-old daughter still wears sunglasses.

The soft glow of a nearby fish tank is about all the light Mimi Alvarez says she can stand before her skin breaks open.

Hunched over on the living room couch, she describes how she can't sit up long because her bones will "lock up" and the stabbing pain she in her stomach will become worse.

"I really try not to think about it," she says of the sensations radiating through her body. "But I don't know how long I will be able to make it."

The Alhambra resident has lived in almost total isolation since she was 12, when she says a series of escalating symptoms - including light sensitivity, extreme nausea and stabbing pain - began.

She hasn't stepped foot in a regular classroom since the sixth grade, and now few friends stop by. But to those who come to visit the thin, pale teenager, it seems clear something is very wrong.

"Mimi has pretty much been like death warmed over since I've known her," said Claudia Jensen, a pediatrician and instructor at USC's Keck School of Medicine who first met Mimi three years ago when her students were unable to find the cause of Mimi's distress.

"Just to hear these thoughts, they run the gamut," said Mimi's father, Henry Alvarez. "It breaks my heart. This is my daughter. She's lost all her youth."

Living through years of frustration and medical dead ends, the knowledge they could finally be heading for a breakthrough is giving the family some hope.

But the diagnosis is not a sure thing.

Richard MacKenzie, Director of Adolescent Medicine at Childrens Hospital Los Angeles, first suspected porphyria after Mimi was admitted to his hospital, complaining of "vague symptoms" that seemed to line up with the disease.

While an initial urine sample tested positive for slightly elevated levels of porhpins, the levels were not high enough for a diagnosis of porphyria. And every subsequent tests for the condition has come back negative.

"There is no doubt that she has real symptoms," he said. "But the tests don't bear them out."

Porphyria support group Web sites are filled with stories of people who went years without getting a positive test. Few labs nationwide are capable of the conducting the test, and operator error, contamination and even administering the test at the wrong time or while a patient is taking nutrition through an IV (like Mimi) may produce false results.

MacKenzie said it's possible. "I'm not pooh-poohing it," he said. "I'm willing to keep an open mind."

A genetic test could offer the family a definitive answer. The Alvarezes set their sights set on a Harvard University researcher in Boston, who is possibly the only person in the country capable of administering such a test.

Boston had another key advantage. Mimi is on feeding tubes because she can't keep solids down. Because of reactions to soy that make her skin break and bleed, she is not being fed the necessary fats. Without them she is weak.

"I'm basically starving all the time," Mimi said. Worse, her organs are in danger. Doctors said they will eventually fail if she continues without fat long term.

Boston Children's Hospital is conducting an experimental trial for a fat that doesn't contain soy, and Mimi is assured a spot if she can get there.

With Mackenzie's blessing, the family planned on catching a flight to Boston.

But like with everything else in Mimi's life, it proved not so simple.

Mimi hasn't left her home, and has barely gotten out of bed, in a month. Her skin has closed up and healed, but getting on a commercial plane with its lights and smells and no room for her many feeding tubes, seems impossible to her.

"I just can't handle it," she said.

Henry Alvarez spends his nights surfing the Internet for nonprofit flight groups that can do the medical transport on a private plane.

He has found groups that offer medical flights at a discounted rate - from $17,000 to $25,000 across the country. With medical bills pouring in each month, it's money they don't have.

"We've pretty much tapped out our house, all our assets," he said.

Mimi's story is now spreading throughout the San Gabriel Valley, and fundraising efforts are under way. The Praise Chapel in Whittier, where the Alvarez family attends church, has been collecting donations and the Elks in Montebello are planning a fundraiser.

In La Puente, Mayor Louie Lujan helped the family open an account for donations.

But fundraising is slow going. Henry Alvarez has taken to soliciting in front of grocery stores.

"I feel like a beggar out there," he said. "But I have to do it."

What exactly is wrong with Mimi has been a mystery from the outset. But the Alvarez family has come to believe she has a form of porphyria - a little-known condition for which there are only 10 specialists nationwide.

Either inherited or acquired, the disease is categorized by a breakdown of enzymes in a part of the body known as the porphyrin pathway. It can manifest itself a variety of ways, causing symptoms like abdominal pain, dark-colored urine, extreme sensitivity to light or smells, skin that blisters or rashes with exposure to the sun - and in some cases paralysis, mental disorders and hallucinations.

It's an explanation they've arrived at after six years parading from specialist to specialist in between lengthy hospital stays.

Doctors first suggested Mimi suffered from anorexia when she dropped to 117 pounds from 236 during three months when she said she couldn't keep food down in 2003. Others believed she was making herself sick for attention, and at least one psychiatrist told her parents they suspected them of making her ill - a mental condition known as Munchausen syndrome. Her family strongly refutes the accusations.

Draconis Blackthorne, shadowgram, Dracomet

Blame Satan! Hedges Sees Hypocrisy, Violence on Christian Right

Blame Satan! Hedges Sees Hypocrisy, Violence on Christian Right
By Susan Antilla

Feb. 6 (Bloomberg) -- Forget Disney World and Epcot Center. You haven't seen anything until you've seen the Creation Museum set to open in Petersburg, Kentucky, this year.

It has a theater with seats that shake and machines that spray mist as God's six-day creation marathon is re-enacted. Museum scientists weave a yarn that has humans and dinosaurs popping up on the very same day -- the sixth, to be exact, which is when the Bible says God made all the land animals.

If you're studying up after your visit, creationists have answers to all your questions. Wondering how God managed to say ``Let there be light'' on Day One without making the sun until Day Four? Hey, no problem -- he's God, after all, so he made a temporary light to cover things until the official Day Four unveiling.

Chris Hedges recounts these and other, more alarming examples of the work of evangelicals in ``American Fascists: The Christian Right and the War on America.'' Hedges, a former reporter for the New York Times and a Harvard Divinity School graduate, focuses on the evangelical movement known as dominionism, in his view a dangerous force that manipulates followers into a faith that is political, intolerant and loaded with the potential for violence.

His firsthand reporting, exposing manipulative preachers who often target recruits precisely because they are down and out and thus more susceptible to conversion, is the strength of the book. Its weakness is his sweeping interpretations of what it all means. Too often he foresees frightening consequences from the machinations of born-again Christians without connecting the dots to show just how they might develop.

Targeting the Vulnerable

The you-are-there scenes from the Christian Right, though, are eye-openers. Hedges attends a seminar for evangelists where he is taught to manipulate and deceive: Target the vulnerable -- people dealing with divorce, death or other crises are more susceptible. Go into a person's home and come up with any compliment. Hide your Bible: ``Don't show your gun until you're ready to shoot it.''

And when challenged on doctrine, blame Satan! That way any tough question can be turned on its head with the accusation that the devil has planted it.

Hedges sees evangelism as a powerful movement scooping up converts who will accept whatever they're told, and it frightens him. It also irks him that dominionists insist, on the one hand, that the Bible be interpreted literally and then, on the other, decide selectively which parts to interpret -- to say nothing of redefining good and evil as they wish.

Liberalism Versus Scripture

Thus, according to the schoolbooks of one major Christian publisher, African religious beliefs are ``false'' and Hinduism is ``pagan'' and ``evil.'' Those of another define ``liberal'' as ``referring to philosophy not supported by scripture'' and ``conservative'' as ``dedicated to the preserving of scriptural principals.''

Just outside an anti-gay conference in Boston, the author stopped beside a pickup truck plastered with homophobic slogans, including a graphic of two men kissing with a red interdiction circle superimposed over it. Across the top of the truck were printed the words ``Stop the Insanity.''

But whose?

``American Fascists'' is published by the Free Press (254 pages, $25).

(Susan Antilla is a columnist for Bloomberg News. The opinions expressed are her own.)

To contact the writer of this story: Susan Antilla in New York at santilla@bloomberg.net