Girl’s Disease Turning Skin to Stone

Friday, August 01, 2008
Fox News

11 year old girl suffers from rare health condition that causes one’s skin to turn to stone…….
[This is a rare disease I have never heard of but certainly can image how hard it must be on her parents to watch and the digression of their daughter’s condition…. ]   

Young girl’s skin is turning to stone as she suffers from a rare, incurable disease called acute systemic scleroderma, Scotland’s Daily Record reported Thursday.

The autoimmune disorder means Hope Barrie’s body produces too much collagen, causing her skin to tighten and harden.

It is difficult for the 11-year-old from Tarbolton, Ayrshire, Scotland, to move around, and she has already had to give up one passion.

“I loved playing the violin and it would be amazing to start playing again,” Hope said.

Hope suffers from the systemic version of the disease, which starts out with poor circulation in the fingers and progresses to thickening of the skin, according to the International Scleroderma Network’s Web site. The disease may even spread to her organs.

As the disease progresses, the hardening of the skin makes it difficult to move fingers and toes, and open the mouth. It also can form scar tissue around the internal organs.

In America, the disease affects about 300,000 individuals [ which in my opinion is too many not be aware of ] many of whom are females between the ages of 30- to 50-years-old at the disease’s onset, according to the International Scleroderma Network.

Hope’s family told the newspaper they’re hoping that pioneering stem cell treatments being done in the U.S. may also help Hope, who is currently undergoing chemotherapy to fight the disease.

Although there is no cure for scleroderma, researchers at Virginia Mason Research Center and Duke University Medical Center are working with a stem cell procedure that has been shown to reverse the symptoms of the disease.

Emily Woods, of Plano, Texas, had the experimental procedure in 2006, according to a New York Times article. Within six months, the 87-pound mother — who had been close to death — was back on her feet and enjoying her daughter, the article said.

The transplant process involves doctors removing the stem cells from a patient’s own blood cells and returning them to the body, where it is hoped they will regenerate.

Click here to read the full story from the Daily Record

[ I know I came across “Gastroparesis” when a good friend of mine called and told me she had been diagnosed with this condition and I was just recently diagnosed with a condition called “hyperparathyroidism”.  It just makes you wonder how many diseases there are that we don’t know about.  I’m thinking of starting a blog on rare conditions that need public awareness.  If anyone would like to help me work on this project just let me know……flagranny2 ]

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Video Shows Woman Ignored While Dying in New York Mental Hospital

Tuesday, July 01, 2008
Article from Associated Press as reported by FoxNews

After watching the video I was in disbelief that someone actually let this happen with no conscience at all.  I guess by now I shouldn’t be shocked by what people allow to happen when they could have helped but something like this makes me feel ashamed to think there are people that can ignore a situation like this………flagranny2

 

BROOKLYN, N.Y. —  Stunning video from a surveillance camera at a Brooklyn hospital shows a woman dying on the floor of a psychiatric emergency room while staffers ignore her.

The video was released by lawyers suing Kings County Hospital alleging neglect and abuse of mental health patients at the medical facility.

Click here to watch the video.

(when you click on this vide it will take you to the one or several ones taken from different views however the one that was originally posted that I first saw closer resembels the 3rd one down on the list at the right where she was completely ignored.) …flagranny2

The video shows the 49-year-old woman falling out of her chair at about 5:30 a.m. June 19, then lying face-down on the floor and thrashing around before going still. The woman died.

People nearby, including two security guards, do nothing to help. An hour passes before a fellow patient finally gets the attention of a staffer.

The tape also suggests that staff at the hospital might have faked medical charts belonging to the victim, Esmin Green, to cover her lack of treatment, the New York Daily News reported.

Her medical chart claims the Jamaica native got up to walk to the bathroom when she was actually writhing on the floor, the News reported. The records also have Green sitting quietly in her chair when she was already dead.

“Thank God for the videotape because no one would have believed this could have happened,” Donna Lieberman, executive director of the New York Civil Liberties Union, told the Daily News.

The agency that runs the municipal hospital — the city’s Health and Hospitals Corp. — fired six staffers, including the two security guards.

Click here for more on this story from the New York Daily News.

‘Elephant Man’ Refuses to Hide From Facial Deformity

Friday, May 30, 2008
news.com.au

O’Neal, of Kirkland, Wash., said he knows his deformity is shocking — but he refuses to hide like other people with his disorder. 

[This is an awesome and heartwarming article I came across.  The admiration I have for this man is tops and I truly commend him for doing what he does as you will see if you click on the link to see the photo.  I must warn you it is quite a shocking picture and probably not for the faint of heart but the article that is included with the picture shows such compassion for someone who has not let his physical disease/appearance hinder him in anyway…flagranny2] 

James O’Neal compares himself to John Hurt’s character in the 1980 film “The Elephant Man.”

A genetic disease known as neurofibromatosis has left O’Neal’s face horribly disfigured, but several surgeries may be able to reconstruct his facial features.

Click here to see a photo of O’Neal.

Although it was at one time believed that Joseph Carey Merrick, the subject of the 1980 film, also suffered from neurofibromatosis, he actually suffered from Proteus syndrome, a congenital disorder that causes skin overgrowth and atypical bone development, often accompanied by tumors over half the body.

Gina Agiostratidou, a biologist and scientific program manager for the Children’s Tumor Foundation in New York, told FOXNews.com that there are three types of neurofibromatosis: NF1, NF2 and schwannomatosis.

All three are caused by deletions or mutations of certain genes. NF1, which is the type suffered by O’Neal, is characterized by large benign tumors that grow on the cranial nerves, face, brain and spinal cord. The disease occurs in about 1 out of every 3,000 births and is more prevalent than cystic fibrosis, according to the Children’s Tumor Foundation.

NF2 is much rarer, occurring in 1 out of 25,000 births. It causes tumors to grow on the cranial and spinal nerves, as well as both auditory nerves. It often results in hearing loss beginning in the teens and 20’s.

Researchers know very little about schwannomatosis. It occurs in about 1 out of every 40,000 births and symptoms differ greatly among sufferers, according to the foundation.

O’Neal, of Kirkland, Wash., said he knows his deformity is shocking — but he refuses to hide like other people with his disorder.

“I just tell people this is who I am, it’s the way I am,” O’Neal told KOMONews.com. “If you don’t like me, you don’t like me.”

But O’Neal — who works as a cashier at Kingsgate Safeway — is well-liked by his customers.

In fact, his regular customers said O’Neal is an “inspiration” and they have started raising money for his surgeries, since insurance likely will not cover the full cost.

“He is an amazing man, and we love him,” said customer Aubrey Richins. “He’s the kind of person that makes your day.”

At the moment, there is no cure for neurofibromatosis, Agiostratidou said.

“There are so many different manifestations of the disease,” she explained. “Surgery is usually a last resort because it does affect the nerves and removing the tumor can create paralysis of the nerve. Currently, the treatment includes chemotherapy (and then) surgery, and we are working on clinical trials for different medications, but it is still very early for us.”

Contributing: Marrecca Fiore, FOXNews.com health editor

 

DA: Wisconsin Parents Who Prayed as Daughter Died to Face Murder Charges

Monday, April 28, 2008

Madeline — called Kara by her parents — died Easter Sunday at the family’s rural Weston home. An autopsy determined she died from undiagnosed diabetic ketoacidosis, an ailment that left her with too little insulin in her body.

WESTON, Wis. —  Parents who prayed as their 11-year-old daughter died of untreated diabetes will be charged with second-degree reckless homicide, the Marathon County district attorney said Monday.

“It is very surprising, shocking that she wasn’t allowed medical intervention,” District Attorney Jill Falstad said.

She announced the charges Monday during a news conference at the Everest Metro Police Department with Police Chief Dan Vergin. Vergin has said Dale and Leilani Neumann told investigators their daughter Madeline last saw a doctor when she was 3 to get some shots.

The couple face up to 25 years in prison if convicted.

The couple and their lawyer did not immediately return messages left by The Associated Press.

Falstad said the Neumanns have cooperated with investigators and are not under arrest. They have agreed to make an initial court appearance Wednesday, she said.

For the complete story click [ Here ]

Zach’s Story: Little Boy’s Courageous Battle Against Old Age

Monday, March 17, 2008

FRANKFORT, Ky. —  If he’s lucky, Zach Pickard will live past the age of 13.

Zach, now 13 months old, suffers from Hutchinson-Gilford progeria syndrome, a disease that accelerates the aging process when the child is 18 to 24 months old. Children with this syndrome die of heart disease at an average age of 13 after aging at a rate six to eight times faster than an average person.

About 100 cases have been formally identified in medical history, and the odds of being diagnosed with it are roughly one in 8 million. For now, Zach is like any other baby, learning to walk, say words like mama and papa and making his family laugh with funny faces.

With his messy blond hair, big blue eyes and infectious smile, Zach attracts attention wherever he goes. Zach was 2 months old when his parents realized something wasn’t right.

Click here for the full story which includes a link related about Hutchinson-Gilford progeria syndrome

After Sanctions, Doctors Get Drug Company Pay

If you know of anyone who is in a clinical drug program this is something they should be made aware of. I know I would be very cautious in choosing one after reading this article.

I think we all are aware that doctors get payback from the drug companies for recommending their product but this goes above and beyond those standards of paybacks and can be more harmful than good. End results can be fatal. An important note to remember is that a lot of the doctors conducting these trial are doctors who have received disciplinary action for one reason or another.

Published: June 3, 2007

A decade ago the Minnesota Board of Medical Practice accused Dr. Faruk Abuzzahab of a “reckless, if not willful, disregard” for the welfare of 46 patients, 5 of whom died in his care or shortly afterward. The board suspended his license for seven months and restricted it for two years after that.

 

But Dr. Abuzzahab, a Minneapolis psychiatrist, is still overseeing the testing of drugs on patients and is being paid by pharmaceutical companies for the work. At least a dozen have paid him for research or marketing since he was disciplined.

Medical ethicists have long argued that doctors who give experimental medicines should be chosen with care. Indeed, the drug industry’s own guidelines for clinical trials state, “Investigators are selected based on qualifications, training, research or clinical expertise in relevant fields.” Yet Dr. Abuzzahab is far from the only doctor to have been disciplined or criticized by a medical board but later paid by drug makers.

An analysis of state records by The New York Times found more than 100 such doctors in Minnesota, at least two with criminal fraud convictions. While Minnesota is the only state to make its records publicly available, the problem, experts say, is national.

One of Dr. Abuzzahab’s patients was David Olson, whom the psychiatrist tried repeatedly to recruit for clinical trials. Drug makers paid Dr. Abuzzahab thousands of dollars for every patient he recruited. In July 1997, when Mr. Olson again refused to be a test subject, Dr. Abuzzahab discharged him from the hospital even though he was suicidal, records show. Mr. Olson committed suicide two weeks later.

In its disciplinary action against Dr. Abuzzahab, the state medical board referred to Mr. Olson as Patient No. 46.

“Dr. Abuzzahab failed to appreciate the risks of taking Patient No. 46 off Clozaril, failed to respond appropriately to the patient’s rapid deterioration and virtually ignored this patient’s suicidality,” the board found.

In an interview, Dr. Abuzzahab dismissed the findings as “without heft” and said drug makers were aware of his record. He said he had helped study many of the most popular drugs in psychiatry, including Paxil, Prozac, Risperdal, Seroquel, Zoloft and Zyprexa.

The Times’s examination of Minnesota’s trove of records on drug company payments to doctors found that from 1997 to 2005, at least 103 doctors who had been disciplined or criticized by the state medical board received a total of $1.7 million from drug makers. The median payment over that period was $1,250; the largest was $479,000.

The sanctions by the board ranged from reprimands to demands for retraining to suspension of licenses. Of those 103 doctors, 39 had been penalized for inappropriate prescribing practices, 21 for substance abuse, 12 for substandard care and 3 for mismanagement of drug studies. A few cases received national news media coverage, but drug makers hired the doctors anyway.

The Times included in its analysis any doctor who received drug company payments within 10 years of being under medical board sanction. At least 38 doctors received a combined $140,000 while they were still under sanction. Dr. Abuzzahab received more than $55,000 from 1997 to 2005.

Drug makers refused to comment, said they relied on doctors to report disciplinary or criminal cases, or said they were considering changing their hiring systems.

Asked about the Minnesota analysis, the deputy commissioner and chief medical officer of the Food and Drug Administration, Dr. Janet Woodcock, said the federal government needed to overhaul regulations governing clinical trials and the doctors who oversaw them.

“We recognize that we need to modernize the F.D.A. approach in keeping people safe in clinical trials,” Dr. Woodcock said.

Drug makers are not required to inform the agency when they discover that investigators are falsifying data, and indeed some have failed to do so in the past. The F.D.A. plans to require such disclosures, Dr. Woodcock said. The agency inspects at most 1 percent of all clinical trials, she said.

Karl Uhlendorf, a spokesman for the Pharmaceutical Research and Manufacturers of America, said the trade group would not comment on The Times’s findings.

The records most likely understate the extent of the problem because they are incomplete. And the Minnesota Board of Medical Practice disciplines a smaller share of the state’s doctors than almost any other medical board in the country, according to rankings by Public Citizen, an advocacy group based in Washington.

Dr. David Rothman, president of the Institute on Medicine as a Profession at Columbia University, said the Times analysis revealed a national problem. “There’s no reason to think Minnesota is unique,” Dr. Rothman said.

“Clinical trial investigators must be culled from only the finest physicians in the country,” he said, “since they work on the frontiers of new knowledge. That drug makers are scraping the bottom of the medical barrel is an outrage.”

Read the full story

 

Florida Woman Who Lost Arms, Legs During Birth Works for Answers

Sunday, May 20, 2007
Fox News

claudia-mejia.jpgSANFORD, Fla. — Claudia Mejia cannot give the hugs most mothers bestow on their children. Nor can she grab her 2-year-old son by the hand or place him on her lap. She cannot tuck her 9-year-old into bed or walk him to the school bus stop.

Motherhood for Mejia, 25, was redefined two years ago when a medical nightmare left her without arms and legs. She contracted a flesh-eating bacteria around the time she gave birth to her youngest son, Matthew, at South Seminole Hospital in Longwood forcing doctors to amputate her limbs to save her life.

The horror that began then still unfolds for the disfigured mother, who has pleaded with hospital officials for two years to turn her medical records over — something a judge just ordered.

She and her lawyers believe she either got the infection — “group A strep” — at the hospital or its doctors and nurses failed to quickly and properly treat it. They are suing the hospital and its parent company, Orlando Regional Healthcare System, seeking unspecified damages.

“I just want the truth,” Mejia said.

Orlando Regional, however, maintains Mejia’s infection was acquired elsewhere and that she was treated properly. In 187,000 births since 1988 at the nonprofit hospital system, comprised of seven medical facilities, Mejia is the only reported case of invasive group A strep, Jennings Hurt III, the hospital’s attorney, said.

“Thank God it’s rare,” Hurt said. “The hospital and everybody associated with the hospital have great sympathy for what happened to her. It’s truly tragic.”

Invasive group A strep infects about three people in every 100,000 in the United States each year, said Dr. Dennis Stevens, chief of the Infectious Disease Section at the Veterans Affairs Medical Center in Boise, Idaho. Of those, about a third reach the flesh-eating stage, named necrotizing fasciitis, that often requires amputation, he said. The flesh-eating stage, which destroys muscles, fat, and skin tissue, is fatal in 30 to 70 percent of its victims, Stevens said.

The three days following the April 28, 2005, birth of her son are troubling, Mejia said. After she checked into the Longwood hospital and gave birth to a healthy baby boy at 7 a.m., a fever set in, medical records show.

A nursing document reveals her temperature was high throughout the day, reading 100.6 degrees at one point. Shaking and chills soon followed, and Mejia complained of severe pain in her stomach and a burning sensation when she urinated — all signs of a potential infection, her attorney, Ron Gilbert, said.

The nurses and midwives deny Mejia ever reported any severe pain, Hurt said.

“A 100.6 temperature is very, very, very low grade,” Hurt said. “A person with group A strep with toxic shock syndrome has very high, spiking temperatures. Ms. Mejia’s symptoms were inconsistent with that.”

Diagnosing women with infection after childbirth can be a struggle, Stevens said.

“Doctors have to be able to read between the lines to know if this is the usual pain from just having had a baby or whether there is something else going on,” he said. “One of the hallmarks of these nasty group A strep infections is severe pain.”

As Mejia’s body tried to fight off the deadly bacteria, nurses treated her with minor painkillers Tylenol and ibuprofen, medical officials confirmed.

The hospital’s care was appropriate and reasonable, Hurt said.

The over-the-counter drugs, however, could not fight a disease commonly treated with an aggressive antibiotic regimen delivered intravenously.

The hospital began the process of discharging Mejia the following morning, but her then-boyfriend, Tim Edwards, complained.

“She’s too sick to come home,” he said. Mejia writhed in pain and began to vomit, still with nothing more than mild pain relievers and anti-inflammatory medications, Edwards said.

Doctors finally stepped in the next afternoon and ordered emergency surgery to remove her uterus, but the invasive bacteria had now entered her bloodstream. She went into toxic shock, her kidneys began to shut down and the blood flow to her arms and legs ceased, causing gangrene to set in, records show.

She was rushed to Orlando Regional Medical Center where doctors informed her that she would die if her limbs were not amputated immediately.

“I wanted to die,” she said. “I really wanted to die. I couldn’t go on living without arms and legs.”

It was Edwards, 34, who spent an hour, racing against the clock, convincing her that she still had much to live for.

Edwards recounts his plea to his girlfriend of two years and the mother of his son: “Keep fighting. And when you’re able, marry me.”

Mejia said yes to life and marriage. Doctors amputated her legs above her knees, the left arm above the elbow and her right arm just below the elbow. Eight days later, Mejia and Edwards exchanged vows in the intensive care unit. She wears her wedding band on a chain around her neck.

Two years later, their love and family remain strong.

Edwards, a produce manager at Target in Sanford, begins each day at 4 a.m. He carries his wife, who without limbs weighs 84 pounds, and bathes her. Nine-year-old Jorge, a child from a previous relationship, cooks breakfast for the family — waffles “made with batter, not the kind you put in the toaster,” his mother points out.

At an awards ceremony at Crystal Lake Elementary in Sanford on Tuesday, proud tears streamed down Edward’s face as Jorge, a third grader there, received awards for good behavior and straight A’s.

Family suppers aren’t outdated either.

“We always eat dinner all together. It’s never apart,” Edwards said. “We’re a family united.”

Mejia has learned to brush her teeth, answer the telephone and surf the Internet. She eats by attaching a fork to a fabric band on her right arm above her elbow. She is motivated to walk again with the help of prosthetics.

Edwards asked her to remarry him last year on Christmas Eve.

“Next time she’s going to walk down the aisle,” he said.

Source: FOXNews.com

Photo: WESH.com