Gene therapy restores hearing in deaf mice

(Medical Press) Using gene therapy, researchers at Boston Children's Hospital and Harvard Medical School have restored hearing in mice with a genetic form of deafness. Their work, published online July 8 by the journal Science Translational Medicine, could pave the way for gene therapy in people with hearing loss caused by genetic mutations.

"Our gene therapy protocol is not yet ready for clinical trials—we need to tweak it a bit more—but in the not-too-distant future we think it could be developed for therapeutic use in humans," says Jeffrey Holt, PhD, a scientist in the Department of Otolaryngology and F.M. Kirby Neurobiology Center at Boston Children's and an associate professor of Otolaryngology at Harvard Medical School...

To deliver the healthy gene, the team inserted it into an engineered virus called adeno-associated virus 1, or AAV1, together with a promoter—a genetic sequence that turns the gene on only in certain sensory cells of the inner ear known as hair cells. They then injected the gene-bearing AAV1 into the inner ear, with these findings:

• In the recessive deafness model, gene therapy with TMC1 restored the ability of sensory hair cells to respond to sound—producing a measurable electrical current—and also restored activity in the auditory portion of the brainstem. 
• Most importantly, the deaf mice regained their ability to hear. To test hearing, the researchers placed the mice in a "startle box" and sounded abrupt, loud tones. "Mice with TMC1 mutations will just sit there, but with gene therapy, they jump as high as a normal mouse," says Holt. (The force of their jump was measured by a plate on the floor underneath them; it was detectable at sounds beginning around 80 decibels.)
• In the dominant deafness model, gene therapy with a related gene, TMC2, was successful at the cellular and brain level, and partially successful at restoring actual hearing in the startle test...(continued)

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One in five ‘brain dead’ patients still alive, claims lawsuit

By Hilary White

NEW YORK, October 1, 2012 (LifeSiteNews.com) – At least one in five patients declared “brain dead” and approved as “organ donors” by one organ donation organisation, are in fact still alive and are being killed by the removal of vital organs, a lawsuit filed last week in Manhattan alleges. The suit outlines the ghoulish worst-case scenario, one that was widely dismissed as scaremongering in the early days of the development of organ transplant technology, but which is getting a second hearing amidst growing concerns that coercion and abuse are becoming increasingly common in the highly lucrative transplant business.

Patrick McMahon, a nurse practitioner and Air Force combat veteran, launched the suit in New York alleging that a major organ donation group is using a quota system for obtaining viable organs. He says it is applying pressure on families and doctors to declare patients dead who are, in fact, still alive and could recover.

The New York Organ Donor Network, McMahon says, even hires “coaches” to help obtain consent “notes”. These coaches, the suit contends, are nothing more than sales and marketing experts who teach transplant coordinators to use high-pressure psychological tactics to play on the emotions of vulnerable family members. The suit alleges that employees who failed to make their quotas were fired.

“You’re not there for grief counselling,” he told the Daily Mail, “you’re there to get organs. It’s all about sales—and that’s pretty much a direct quote from the organisation. Counsellors are required to get a 30 per cent consent rate from families.” The top “counsellors” get a Christmas bonus, he added.

McMahon’s suit cites one case in which a 19-year-old car crash victim was struggling to breathe and showing signs of brain activity, but doctors signed off on the “donation.” The suit alleges that Network director, Michael Goldstein, “bullied” staff at the Nassau University Medical Center. It quotes him telling a conference call, “This kid is dead, you got that?”

“I have been in Desert Storm, Iraq and Afghanistan in combat. I worked on massive brain injuries, trauma, gunshot wounds, IEDs. I have seen worse cases than this and the victims recover,” McMahon told the Washington Post.

McMahon was an employee of the Organ Donor Network, but was fired shortly after bringing his concerns to the attention of the CEO. The suit was filed on September 25 in Manhattan Supreme Court and says that on November 4 last year, McMahon spoke with Helen Irving, the president and CEO of the Organ Donor Network. He told her that, “one in five patients declared brain dead show signs of brain activity” when the official notice of brain death is issued.

The suit quotes Irving replying, “This is how things are done.”

The Washington Post quoted Organ Donor Network’s spokesman Julia Rivera saying that though she had not seen the documents, claims of a quota system are “ridiculous.” “There are no quotas.”

McMahon’s suit accuses the Network of “wrongful and illegal practices.” McMahon was fired from his position as a transplant coordinator on November 15th, just days after his alleged conversation with Helen Irving. The stated reason, he was told by the Network, was failure to properly satisfy job requirements and inefficiency, accusations he calls “ridiculous” and “totally false.”

“It’s atrocious what’s going on,” McMahon told Staten Island Live. “These individuals that aren’t brain dead can’t speak for themselves. The family members aren’t experts. I’m trying to stop [the network] from doing this,” he said.

The suit alleges that in another case a man admitted to Kings County Hospital in Brooklyn was also showing signs of brain activity, but though McMahon protested, the man was declared brain dead and his organs were removed. In a third case cited, a woman was declared brain dead after being admitted to Staten Island University Hospital for a drug overdose. McMahon says when she was having her organs removed, he noticed she was being administered a “paralyzing anesthetic” drug to stop her body from jerking on the operating table.

He told the Daily Mail, “She was having brain function when they were cutting into her on the table. He had given her a paralyser and there’s no reason to give someone who is dead a paralyser.”

On being confronted, the hospital staffer administering the drug told McMahon that he had been told to do it because she was moving as her chest was being cut open. “A paralyser only paralyses you, it does nothing for the pain,” McMahon told the Mail.

In that case, McMahon said, surgeons “took everything”. “They took her eyes, her joints. She was right there when I was having the conversation. They were inserting the plastic bones where the real ones had been.”

In response to McMahon’s protests, Network staff told the hospital he was “an untrained troublemaker with a history of raising frivolous issues and questions.”

“Despite these facts, over [McMahon’s] vehement objections, NYODN continued to process the female patient and allowed the life-ending surgery to go forward,” the court documents say. “NYODN’s actions are the direct cause for the female patient’s premature death.”

Ethicists continue to warn that money, not altruism, is the driving force behind organ transplants that have become a multi-billion dollar, global industry. In 2008, after the Vatican’s own Pontifical Academy for Life co-sponsored a high-profile international conference on organ transplantation, many in the pro-life community protested that no mention was made by any speaker of the ethical concerns over coercion or death criteria.

But after the vociferous international outcry, Pope Benedict XVI himself issued a stern warning in his address to the conference attendees, saying that the primary concern must be that organ transplantation does not devolve into a choice of one life over another.  “Informed consent is the precondition of freedom, so that the transplant has the characteristic of a gift and cannot be interpreted as an act of coercion or exploitation,” the pope said. The medical community must reject the illicit trade in organs “which often affect innocent people such as children” and the “utilitarian criteria” for donation. These must be, he said, “strongly condemned as abominable.”

In 2008, the New England Journal of Medicine printed an article that frankly argued that “brain death” is a sham. The article, co-authored by Dr. Robert D. Truog, a professor of medical ethics and anesthesia (pediatrics) in the Departments of Anesthesia and Social Medicine at Harvard Medical School, said the scientific literature does not support the criteria for ‘brain death’ and ‘cardiac death’ as being real death. “Although it may be ethical to remove vital organs from these patients, we believe that the reason it is ethical cannot convincingly be that the donors are dead,” the article said.

So open has the “brain death” secret become in medical circles that some are urging that such criteria simply be dropped. Dr. Neil Lazar, director of the medical-surgical intensive care unit at Toronto General Hospital, Dr. Maxwell J. Smith of the University of Toronto, and David Rodriguez-Arias of Universidad del Pais Vasco in Spain, admitted at a major conference that the pretense should be ended and that organs should be allowed to be removed from “dying” or “severely injured” patients. This more “honest” approach, they said, would avoid the problems created by purely ideological definitions of death that are known to be mere pretexts to expand the organ donor pool.

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• One in five ‘brain dead’ patients still alive, claims lawsuit 

Related:

• Dad rescues ‘brain dead’ son from doctors wishing to harvest his organs – boy recovers completely
• Critical organ donation warnings and guidance in new pamphlet: “Do not do an Apnea test!”

Honey could be a wonder drug

From correspondents in Ottawa

September 25, 2008 02:45am

HONEY, used for generations to soothe sore throats, could soon be substituted for antibiotics in fighting stubborn ear, nose and throat infections, according to a new study.

Ottawa University doctors found in tests that ordinary honey kills bacteria that cause sinus infections, and does it better in most cases than antibiotics.

The researchers have so far tested manuka honey from New Zealand, and sidr honey from Yemen.

"It's astonishing," researcher Joseph Marson said of bees' unexplained ability to combine the nectar of flowers into a seemingly potent medicine.

The preliminary tests were conducted in laboratory dishes, not in live patients, but included the "superbug" methicillin-resistant Staphylococcus aureus or MRSA, which is highly resistant to antibiotics.

In upcoming human trials, a "honey rinse" would be used to "flush out the goo from sinus cavities," said Marson.

The two killed all floating bacteria in liquid, and 63-91 per cent of biofilms - micro-organisms that sometimes form a protective layer in sinus cavities, urinary tracts, catheters, and heart valves, protecting bacteria from normal drug treatments and often leading to chronic infections.

The most effective antibiotic, rifampin, killed just 18 percent of the biofilm samples in the tests.

"As of today, nobody is sure what in the honey kills the bacteria," Marson said, noting that "not all honeys have the same potency" and calling for more research to determine the mechanism behind the healing.

Canada's clover and buckwheat honey did not work at all.

Previous studies have shown honey's healing properties on infected wounds.

The results of the study were presented this week at the annual meeting of the American Academy of Otolaryngology-Head and Neck Surgery, in Chicago.

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Robert D. Novak: My Brain Tumor

WASHINGTON, D.C. -- The main reason I am writing this column is that many people have asked me how I first realized I was suffering from a brain tumor and what I have done about it.

But I also want to relate the reaction to my disease, mostly compassionate, that belies Washington's reputation.

The first sign that I was in trouble came on Wednesday, July 23, when my 2004 black Corvette struck a pedestrian on 18th Street in downtown Washington while I was on my way to my office.

I did not realize I had hit anyone until a shirt-sleeved young man on a bicycle, whom I incorrectly thought to be a bicycle messenger, jumped in front of my car to block the way. In fact, he was David A. Bono, a partner in the high-end law firm Harkins Cunningham. The bicyclist was shouting at me that I could not just hit people and then drive away. That was the first I knew about the accident. Mr. Bono called the police, and a patrolman soon arrived.

After I said I had no idea I had hit anyone until they flagged me down and informed me, Mr. Bono told The Washington Post, "I would not believe that." Fortunately, the investigating officer, P. Garcia, was a policeman who listened and apparently believed me. While Mr. Bono and other bystanders were taking on aspects of a mob, shouting "hit-and-run," Officer Garcia issued a right-of-way infraction against me, costing me $50, instead of a hit-and-run violation that would have been a felony. Following Officer Garcia's instructions, I promptly paid the $50 fine at Third District Police Headquarters in Northwest Washington, in cash and in person.

Officer Garcia's justification in believing me was soon confirmed by the diagnosis of my brain cancer, in which I have lost not only left peripheral vision but nearly all my left vision, probably permanently. Several people have asked me whether the person I hit was crossing in front of me on my left. I answer, "I never saw him."

The person I hit, identified by police as Don, with no fixed address, was taken to George Washington University Hospital, where police said, "There are no visible injuries."

On the next day, Thursday, July 24, there were more clues that something was seriously wrong. I lost my way to my dentist's office in Montgomery County and never found it. I also had trouble finding my way back to my office. After returning from a speaking engagement in North Carolina on Friday, I found it difficult locating my office in the 13-story building where I have been a tenant since 1964.

My wife Geraldine and I left Washington Saturday to spend the weekend with our daughter, Zelda, and her husband, Christopher Caldwell, and their children at their summer house at Manchester-by-the-Sea, Mass. When Geraldine noticed that I was having trouble following her in the Boston airport, she suggested I go to a hospital emergency room. I always resist such suggestions and did so this time, but fortunately Zelda prevailed. The CT scan at Salem Hospital showed a brain mass. I returned to the summerhouse and went into seizure the next day.

When Zelda said to call 911, I again resisted, but she again prevailed. I promptly suffered another seizure in the ambulance, the second of three seizures that day. I gained admittance to the high-quality Brigham and Women's Hospital in Boston, which has an excellent oncology staff. A biopsy was performed, which showed a large, grade IV tumor. In answer to my question, the oncologist estimated that I had six months to a year to live. Being read your death sentence is like being a character in one of the old Bette Davis movies.

I believe I was able to withstand this shock because of my Catholic faith, to which I converted in 1998.

I then called Dr. Donald Morton of the John Wayne Cancer Institute in Santa Monica, Calif., who removed a cancer from my lung in 1994 and has been a friend and close medical advisor. He told me that different people react to serious cancers in different ways and reminded me that I was a three-time cancer survivor.

Dr. Morton recommended Dr. Allan H. Friedman, a master surgeon who is chief of neurosurgery at the Duke University Medical Center.

After studying my CT scan and MRI, Dr. Friedman said a resection -- that is, a removal of the tumor -- was possible by surgery. Dr. Friedman had performed a similar operation this summer on Sen. Edward M. Kennedy of Massachusetts.

In today's world, it is up to the "informed patient" to make many decisions affecting treatment. Dr. Morton recommended that I go ahead with surgery by Dr. Friedman.

My dear friend, the Democratic political operative Bob Shrum, asked Sen. Kennedy's wife, Vicki, to call me about Dr. Friedman. I barely know Mrs. Kennedy, but I have found her to be a warm and gracious person. I have had few good things to say about Teddy Kennedy since I first met him at the 1960 Democratic National Convention in Los Angeles, but he and his wife have treated me like a close friend. She was enthusiastic about Dr. Friedman and urged me to opt for surgery at Duke, which I did.

The Kennedys were not concerned by political and ideological differences when someone's life was at stake, recalling at least the myth of milder days in Washington. My long conversation with Vicki Kennedy filled me with hope.

The irony of my going to Duke to save my life can only be appreciated by somebody who knows that I am a fanatic University of Maryland basketball fan with no use for the Duke Blue Devils and their student basketball fans, who certainly have not turned the other cheek toward me.

The ingenious taunts by the students at Duke's Cameron Indoor Stadium are usually directed against opposing players, but I am one fan who also has been the target of the "Cameron Crazies."

During my last visit there to watch a game won by Maryland, students raised a placard with two pictures: one of Benedict Arnold and one of me. "Two Traitors, " said the headline.

But I was treated with immense courtesy and skill by the great Duke neurosurgical team. Dr. Friedman operated on me for over four hours, starting at 7:30 a.m. on Friday, Aug. 15. He later showed me before-and-after pictures, revealing that the 3-by-1.5-inch tumor had been removed. Of course, cancer cells remain, requiring a rigorous regimen of radiation and chemotherapy, managed by the Duke team and conducted at the George Washington University Hospital in Washington.

Al Hunt, who has become a close friend, though we disagree about almost everything, says it will be very difficult for me to inveigh against Duke in the future. I do believe he is correct. Al and his wife, Judy Woodruff, have been staunch pillars of support during this ordeal and helped arrange our living accommodations at Duke.

I am now at home in Washington, awaiting further therapy. Dr. Friedman recommended that I try to get back to at least parts of my normal life. He suggested reading, but also that I try to write columns, which is the reason I've composed this piece.

There are mad bloggers who profess to take delight in my distress, but there's no need to pay them attention in the face of such an outpouring of good will for me. I had thought 51 years of rough-and-tumble journalism in Washington made me more enemies than friends, but my recent experience suggests the opposite may be the case.

But Joe and Valerie Wilson, attempting to breathe life into the Valerie Plame "scandal," issued this statement: "We have long argued that responsible adults should take Novak's typewriter away. The time has arrived for them to also take away the keys to his Corvette."

Thanks to my tumor, the Wilsons have achieved half of their desires. I probably never will be able to drive again, and I have sold the Corvette, which I dearly loved. Taking away my typewriter, however, may require modification of the First Amendment.

Support for me and promises of prayers sent for me poured in from all sides, including political figures who had not been happy with my columns. I'm told that President George W. Bush has not liked my criticism, particularly of his Iraq war policy. But the president is a compassionate man, and he telephoned me at 7:24 a.m. on August 15, six minutes before I went into surgery. The conversation lasted only a minute, but his prayerful concern was touching and much appreciated.

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Fish oil appears to help against heart failure

MUNICH, GERMANY (AP) - Fish oil supplements may work slightly better than a popular cholesterol-reducing drug to help patients with chronic heart failure, according to new research released Sunday...

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