Angelina Jolie's mother had breast cancer and died of ovarian cancer, and her maternal grandmother also had ovarian cancer — strong evidence of an inherited, genetic risk that led the actress to have both of her healthy breasts removed to try to avoid the same fate, her doctor says.

Jolie, 37, revealed on Tuesday that she carries a defective BRCA1 gene that puts her at high risk of developing breast and ovarian cancer. She had mastectomies in February followed by reconstruction with implants in April, Dr. Kristi Funk said in an interview with The Associated Press.

Funk treated Jolie at the Pink Lotus Breast Center in Beverly Hills and detailed her care on the center's website. She would not disclose when Jolie learned she carried the faulty gene, which gives a woman up to an 87 percent lifetime risk of developing breast cancer and up to a 54 percent chance of ovarian cancer.

"This family history would certainly meet any insurance carrier's criteria to cover genetic testing," Funk wrote.

It is unclear whether Jolie will have her ovaries removed, although she wrote in her op-ed piece in the New York Times that she "started with the breasts" because they posed the highest cancer risk.

Removing the ovaries is often advised for women with such gene mutations, said Dr. Charis Eng, a medical geneticist and cancer specialist at the Cleveland Clinic who had no role in Jolie's care.

"We usually say 'try to have your kids'" and then have your ovaries removed by age 40, Eng said. It's not possible to remove every speck of breast tissue, but removing the breasts and ovaries leaves very little behind that could develop cancer, so it dramatically lowers a woman's risks, she said.

On the surgery center's website, Funk described Jolie's three operations, which were done through the crease underneath each breast. Jolie's partner, actor Brad Pitt, "was on hand to greet her as soon as she came around from the anesthetic, as he was during each of the operations," Funk wrote.

On Feb. 2, Jolie had a procedure aimed at preserving the nipples, which usually are removed when a mastectomy is done to treat breast cancer. Half of the skin is lifted from the surface of the breast tissue and a small disc of tissue is taken to be checked for cancer.

The tissue proved healthy and on Feb. 16 she had the two mastectomies. Doctors also took an unusual step: injecting dye to determine which lymph nodes in her arms were draining fluid from the breasts. Those nodes would be most likely to contain cancer if any turned out to be lurking in the breast, Funk explained.

When a preventive mastectomy is done, "there's a 2 to 8 percent chance" of finding cancer, even though there was no indication of cancer beforehand, Funk said. Stitches or a tiny clip can be placed to show the location of these key "sentinel" lymph nodes in case Jolie ever were to develop cancer in the future and those nodes would need to be checked again.

Also during this operation, doctors placed a tissue expander, a balloon-like device that is slowly inflated with saline to stretch the skin and make room for a permanent implant. Even though the implant could have been done at the time of the mastectomy, Jolie chose the two-step procedure to optimize the final cosmetic appearance.

Four days after her mastectomies, "I was pleased to find her not only in good spirits with bountiful energy, but with two walls in her house covered with freshly assembled storyboards for the next project she is directing," Funk wrote on the website.

On April 27, doctors did her reconstruction, using a newer teardrop-shaped implant plus sheets of cadaver skin, which "creates like a sling under the implant" to give a more natural look, Funk said.

The website describes how women with gene mutations that raise their risk for cancer are monitored starting at age 18, but Funk said she could not disclose when Jolie was tested and learned she had the BRCA1 gene. About 5 percent to 10 percent of breast cancers and about 15 percent of ovarian cancers are thought to be due to BRCA gene mutations.

Related:

More opt for preventive mastectomy

Don't judge us, mastectomy patient says

By Kate Kelland
Reuters 

Bereaved parents who do not want to see their dead babies go through a conventional autopsy could in future be offered a less invasive option which uses magnetic resonance imaging and blood tests to establish the cause of death. 

Scientists who investigated using a combination of full body scans and sample tests found this so-called minimally invasive autopsy (MIA) was as effective in determining the cause of death as a conventional procedure, which involves an open dissection of the baby's body to examine the organs.

Since the vast majority of parents whose babies die during or soon after birth currently refuse any autopsy, the researchers suggested the MIA could both improve rates of uptake and reduce parents' distress while offering clear answers.

"Autopsies not only help us to establish the cause of death, but they often play an important role in advancing medical research and knowledge," said Andrew Taylor, a consultant radiologist at London's Great Ormond Street Hospital and University College Hospital who co-led the study.

"If we can find ways to continue to carry them out using less invasive methods, such as post-mortem MRI, we can boost our understanding of the many ways in which the body can go wrong."

In a study published in the journal the Lancet, Taylor and colleague Sudhin Thayyil, a consultant neonatologist, compared the accuracy of a standard autopsy with that of whole-body, post-mortem MRI with or without other minimally invasive tests.

These included blood samples taken by needle, visual examination of the body and genetic and metabolic tests.

The study involved 400 cases of  foetuses, babies and children under 16 years old.

For foetuses and babies younger than a year, the MIA identified the same cause of death as the full autopsy for 92 percent of the cases studied, while in children aged one to 16, the MIA techniques were less accurate, with 54 percent of the two types of autopsies agreeing on cause of death.

The researchers said the difference in accuracy was probably because MRI was good at picking up abnormalities in organ structure or function, which are more likely to be causes of death in young babies, but unable to detect infections, which are more likely to be a cause of death in older children.

Experts say that currently in Britain, some 80 percent of parents whose baby dies shortly after birth refuse consent for a post mortem. This is despite evidence that autopsies find new and useful information in the majority of cases.

In the United States, Thayyil said, rates of autopsy in babies are even lower. 

"In a state of shock and grief, parents are asked if they will consent, and while they desperately want answers about why their baby died, many simply cannot contemplate what a post mortem entails," said Charlotte Bevan of Sands, a charity that campaigns for more research into stillbirth and neonatal death.

"Giving parents the option to have a less invasive but equally informative investigation will not only make the decision easier ... but could lead to an increase in post mortem up-take and vastly improved research into why so many babies are stillborn or die shortly after birth."

Nurse practitioners are staffing retail health clinics, diagnosing and treating ills from strep throat to conjunctivitis. They’re giving flu shots and prescribing drugs. And the influential Institute of Medicine says they should not only work side by side with physicians, but replace them in some cases.

But a survey published on Wednesday shows a huge gap between what nurse practitioners think they can and should do, and what doctors think. And that’s bad news for patients, Karen Donelan of the Mongan Institute for Health Policy at Massachusetts General Hospital says.

“We were surprised by the level of disagreement reported between these two groups of professionals," says Donelan, who led the survey published in the New England Journal of Medicine.

Her team’s survey of 467 nurse practitioners and 505 physicians found both groups agree that nurse practitioners should practice “to the full extent of their education and training.” Where the disconnect comes is just what this training should allow them to do, and how much they should get paid for it.

Only 17 percent pf physicians agreed that nurse practitioners should coordinate a patient’s care as a leader of a “medical home”, versus more than 82 percent of the nurse practioners, the survey found. And only 3.8 percent of doctors felt that a nurse practitioner should be paid the same for providing the same service as a physician, compared to 64 percent of the nurse-practitioners.

“At the core of the controversy is whether nurse practitioners have the education and experience to provide high-quality services and lead clinical practices without supervision by a physician,” Donelan’s team wrote.

The Institute of Medicine tried to settle that question in a 2010 report, saying that nurses can handle much of the strain on the health care system and should be given both the education and the authority to take on more medical duties.

But the American Medical Association, which represents about 120,000 practicing physicians and students, rejected the idea immediately.

"Nurses are critical to the health care team, but there is no substitute for education and training," the AMA said at the time.

The Association of American Medical Colleges estimates that by 2015, the United States will be short about 62,100 physicians. Many experts are looking to the 180,000 nurse practitioners now in the field to help cover the gaps.

When the Robert Wood Johnson Foundation set up a series of meetings among doctors’ and nurses’ guilds to try to smooth out the disagreements in 2011, things looked up until the organizers issued a report urging less hierarchy, says John Iglehart of the journal Health Affairs in a separate commentary in the New England Journal. “The `captain of the ship' notion  … needs to be eliminated, focusing on the patient as the driver of care,” the report read. “A physician, nurse, social worker or other provider may take the lead in a given situation.’

The American Academy of Family Physicians,  American Osteopathic Association and the American Academy of Pediatrics pulled out and the whole attempt collapsed, Iglehart says.

Dr. David Blumenthal and Melinda Abrams of the Commonwealth Fund said the survey confirmed that doctors and nurse practitioners often “inhabit different universes”.  And clearly they have different training and skills. Nurse practitioners, often referred to as advanced practice registered nurses, usually have a four-year bachelor's degree with at least a master's degree and sometimes a doctorate beyond that, depending on the requirements of their state.

But nurse practitioners can fill the growing gap in the supply of health care prviders for the U.S., they argued. “The existing literature shows that nurse practitioners provide care similar to that of physicians with respect to health outcomes, resource utilization, and cost, and the same studies show that nurse practitioners get higher grades than physicians with respect to communication with patients seeking urgent care,” they wrote.

Jan Towers, policy director for the American Association of Nurse Practitioners, saw it coming. “There is nothing surprising there,” she said in a telephone interview. “The disconnect has been there a long time.”

She thinks relationships will improve as doctors and nurse practitioners work together more. A bigger issue, Towers says, is the way the U.S. healthcare system pays for care. “How do we institute value-based purchasing so that we look at outcomes rather than who is performing the task?” she asked.

Once the medical system evolves toward taking care of a patient’s health, instead of the current system of paying for each individual test, treatment or consultation, the differences should even out, she predicts.

Donelan isn’t so hopeful. “Our findings suggest that a substantial number of primary care physicians are unlikely to embrace policy recommendations aimed at further expansion of the roles and supply of nurse practitioners,” her team wrote.

“In particular, physicians' concerns about the likely effect of an expanded workforce of nurse practitioners on several aspects of health care quality need to be addressed in discussions of strategy for the development of the U.S. health care workforce.”

Peter Buerhaus, director of the Center for Interdisciplinary Health Workforce Studies at Vanderbilt University and a registered nurse with a Ph.D who worked on the survey team, agreed.

"It is unsettling that primary care physicians and nurse practitioners, who have been practicing together for several decades, seem so far apart in their perceptions of each other's contributions. I am concerned that these large gaps in perceptions will inhibit efforts to redesign care delivery and to improve the productivity and configuration of the primary care workforce,” Buerhaus said in a statement.

Related:

• Nurses need a more independent role, report argues
• Major doctor shortage coming
• Shortage of family doctors predicted by 2020

It has finally happened – the technique used in 1996 to make Dolly, the world’s first cloned mammal, has finally been used in 2013 to make the world’s first cloned human embryo. Despite a history in the years after Dolly of nuts, crackpots, frauds and charlatans announcing that they had either cloned embryos or cloned babies--who can forget the Raelians with their star fleet uniforms announcing the creation of multiple clone baby births to a credulous press core—we have an announcement that is the real deal. A team of experts in cloning at Oregon Health Sciences University who have extensive experience and success with primate cloning have announced the cloning of human embryos.

This announcement is sure to set off a heated debate about the morality of what they have done and what could be done with cloned human embryos. But while there is some reason for concern, there is more reason for excitement.

The Oregon team has been trying to clone human embryos for many years. Why? Not to produce cloned people but to have a source of stem cells useful for the treatment of diseases.

Those who oppose manipulating embryos to generate stem cells -- and their number is huge -- will be blasting away at what has been achieved.

But before they try to freak you out with terrifying images of clone armies directed by despots (think “Star Wars: Episode II - Attack of the Clones”), the unmourned dead coming back to life via cloning (think Osama bin Laden) or the creation of multiple copies of particularly odd or dangerous people (think of dozens of versions of Lindsay Lohan or Charlie Sheen in your neighborhood), remember that the whole point of cloning research is to come up with stem cells that have the same genetic makeup as the person who needs them.

Stem cells can be obtained from human embryos at fertility clinics. But the cells that are made from them will not match those to whom they might be transplanted to repair macular degeneration, spinal cord injury or diabetes. Through cloning you can take a disabled or sick person's DNA from one of their body cells, insert it into a human egg from which the DNA has been removed, fuse the cell electrically (the technique used in Oregon) and create an embryo from which cells can be grown that are identical matches to what the sick or disabled person needs.

There are certainly crucially important ethical issues that cloning raises.  Should anyone be allowed to try to make people using cloning? Most assuredly not until cloning efforts with animals prove far safer then they currently are. Many animals made via cloning die in utero, are stillborn or have a variety of serious health issues as did Dolly, the first cloned sheep. For now, banning human reproductive cloning—not cloning for stem cell research, as many nations have already done -- ought to be a legislative priority in the U.S. and around the world.

If we are going to need eggs to clone human embryos, then where are they coming from? Most likely in the short run from donors who will have to fully understand what their eggs will be used to create. Whether paid sellers of eggs will be needed in the future remains to be seen, but that is not yet likely to be a problem.

And some will say we don’t need to make cloned embryos to get stem cells because there are other ways to get them. There are other ways but this may prove to be the best way medically to get the regenerative cells that so many could benefit from.

Cloning a human embryo to create stem cells has been a dream for many scientists since Dolly was born. Cloning a human embryo has been a source of ethical nightmares for many theologians, ethicists and scientists since Dolly was born. It has now time to decide if we can manage a technology that holds great promise while assuring those who fear its abuse that their concerns will be fully addressed.

Arthur Caplan, Ph.D., is the head of the Division of Medical Ethics at NYU Langone Medical Center.

Related stories:

Cloning technique produces human stem cells for first time

Ethicist: Fixing genes using cloning technique is worth ethical risk

Oregon Health&Science University

Donor egg held by pipette prior to nuclear extraction.

Researchers say they have finally managed to use cloning technology to make human embryos and grow stem cells from them in the hopes of making perfectly matched grow-your-own tissue transplants.

They used a human egg cell and parts of a human skin cell to grow a very early human embryo, then transformed cells from this ball of cells into beating heart cells and skin cells. The process may eventually help treat a range of diseases, from Parkinson’s to rare inherited conditions, they reported Wednesday in the journal Cell.

The researchers, at Oregon Health & Science University, say their embryos almost certainly could not grow into living human babies or even start a pregnancy – they’re deficient in a key way. But they admit also that they haven’t quite overcome ethical qualms about working with human embryos.

However, the work opens another route to treatments using human embryonic stem cells, the body’s master cells. “These stem cells are kind of very early unprogrammed cells but they have the capacity to become any other cell type,” says Shoukhrat Mitalipov, who led the research.

These cells are very different from so-called adult stem cells, like those taken from bone marrow. Adult stem cells cannot give rise to cells of other tissue types -- blood cells cannot be used to make brain cells, for instance.

Dr. George Daley, a stem cell expert at Harvard Medical School, called it a "beautiful piece of work".

When human embryonic stem cells were first discovered in 1998, scientists immediately dreamed of using cloning technology to help people grow their own organ and tissue transplants, and to use them to study disease. They’d be perfect genetic matches for each patient, meaning an end to a lifetime of taking dangerous immune-suppressing drugs after an organ transplant.

But in the many years since, no lab’s been able to do the work easily. It seems it is much harder to clone a human being than it is to clone a sheep, a frog or a mouse. And then there are the ethical concerns, not only concerns about cloning human beings but over working with human embryos. A federal court has only just ruled in the past year that government funds may be used in the research.

Scientists have found several other routes to harnessing the power of these master cells, which can give rise to any tissue type in the body, from nerve cells to muscle, bone and skin. There are cells taken from embryos left over at fertility clinics – currently being tested as treatments for blindness by a company, Advanced Cell Technology of Massachusetts.

Oregon Health & Science University

Researchers at Oregon Health & Science University have successfully developed a method for converting human skin cells into embryonic stem cells.

Other groups have learned how to “trick” ordinary skin cells into re-modeling themselves into different tissues. These so-called induced pluripotent stem cells, iPS cells for short, might also some day be used to grow transplants perfectly matched to a patient. But again, the technique isn’t easy and there have been many stumbling blocks.

Several other scientists said the science was sound, but said the field had mostly moved on from the pursuit of cloning technology. "IPS cells are easier to produce and have wide applications in research and regenerative medicine, and it remains to be shown whether (cloned embryonic stem cells) have advantages over iPS cells," Daley said by e-mail.

Cloning almost got left in the dust with the work on the other techniques. But the team at OHSU had been perfecting the technique in monkeys, and now they’ve managed to make it work with human cells. The advantage, they say, is that the donated human egg provides fresh and rejuvenating DNA.

The technique they use is called somatic cell nuclear transfer – the same method used to make Dolly, the sheep who was the first mammal cloned from the cell of another adult mammal, in 1996. Scientists remove the nucleus from a normal cell, usually a type of skin cell. They do the same with a human egg cell, then inject the nucleus from the skin cell into the egg.

Various chemical or electrical tricks can be used to start the egg growing as if it had been fertilized by sperm. The method’s been used to make sheep, dogs, horses, and mice – but never human beings.

None of these clones are precise copies because the egg contains an important source of DNA, called mitochondrial DNA. And defects in this DNA cause many diseases, including diabetes and a condition called Leigh syndrome, which causes seizures and dementia.

Mitalipov hopes that replacing the mitochondrial DNA as part of the cloning process might help make tissue that could correct these diseases. His team tested cells taken from a patient with Leigh syndrome, a neurological disorder, and made stem cells using the technique.

“It allows you to produce genetically corrected cells,” he said. “There are a variety of age-related diseases that we believe are caused by acquired mitochondrial mutations.”

Lots of testing lies ahead and because of laws banning the use of federal money to directly make human embryos, Mitalipov’s lab uses private funds instead. But he believes the method cannot be used to make human babies.

“We have been doing it for years in primates and the embryos never implant,” he said. The blastocysts appear to lack a key layer of cells, he said, that give rise to the placenta and that are needed for a normal pregnancy.

Nonetheless, he admits that is unlikely to reassure people who object to experimenting on human embryos. “They’ll say ‘oh, you are just creating a disabled embryo’,” he said.

O. Carter Snead, a bioethicist and professor of law at the University of Notre Dame, called it sad news. “The use and destruction of living human beings – at any stage of biological development – for scientific research is a terrible injustice.  Human cloning for biomedical research is a particularly aggravated form of this harm," Snead said in a statement.

Another barrier --- human eggs are not easy to come by and there are also ethical questions about whether women should be paid to donate their eggs for this kind of research.

The work will almost certainly be used to study diseases in lab dishes at first. But Daley, who heads the bone marrow transplant program at Boston Children's Hospital, said using a patient's own cells offers potentially huge advantages. "A lot of patients don't have an optimal donor," he said. So bone marrow transplants are done only for the patients in the most dire need.

"If we could make every patient their own donor ... we would bypass the transplant barrier," he said. "Everyone could be a donor for themselves."

Related:

• Ethicist: Cloning offers more cause for excitement, not concern
• Supreme Court lets embryonic stem cell research go forward
• Court rules on controversial human research
• Stephen Hawking visits stem cell lab

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No more long monologues for Google co-founder Larry Page. The 40-year-old chief executive says both his vocal cords have some degree of paralysis, which he says was caused by a virus.

Larry Page, Google’s co-founder and chief executive, says the condition that has left his voice hoarse and raspy and made public speaking difficult was caused by a virus.

Page disclosed Tuesday that he has some degree of paralysis in both of his vocal cords. That, he said, causes breathing problems and allows him to speak only in a quiet voice.

“Thankfully, after some initial recovery I’m fully able to do all I need to at home and at work, though my voice is softer than before,” Page said in a written statement. “And giving long monologues is more tedious for me and probably the audience.

At least 5 percent of Americans have experienced paralysis of a vocal cord, although many more probably go undiagnosed, said Dr. Natasha Mirza, a professor of otolaryngology at the Perelman School of Medicine at the University of Pennsylvania.

Paralysis can result from trauma to the cords during surgery or an accident, but many cases are thought to be related to a viral infection that somehow damages the nerves that power the vocal cords, Mirza said.

When everything is working correctly, “we create a voice when the two vocal cords – strands of muscle in the throat – come together forcing air through them,” explained Dr. Martin Hopp, director of the sinus center at Cedars-Sinai Medical Center. “When the cords separate apart, we breathe.”

We each have two vocal cords – one on the right and the other on the left – of the voice box, Hopp said. “Like every other muscle, the vocal cords are driven by nerves and those nerves can be damaged,” he added.

With one cord paralyzed, the other has to work harder to close the gap between them when we want to vocalize, Hopp said. And that is what leads to a weakened voice.

While experts believe that viruses are at the root of about 40 percent of vocal cord paralyses, no one really knows for sure. It’s just a circumstantial case: A person gets a virus and, close on the heels of that, becomes hoarse.

That’s what Page says happened to him. His left vocal cord became paralyzed about 14 years ago after a viral infection. And then, recently, after another virus struck, his right vocal cord lost much of its function.

Page appears to be among the unfortunate ones whose vocal cords don’t recover.

A study published in Laryngoscope in 2008 found that 36 percent of vocal cords fully recover on their own, with another 39 percent experiencing partial recovery. The rest stay paralyzed, like Page’s left vocal cord.

For those with vocal cords that don’t recover, there are options, said Dr. Albert Merati, a professor and chief of the laryngology service at the University of Washington School of Medicine in Seattle. The simplest solution is to “plump up” the paralyzed cord. By doing that, doctors narrow the gap between the paralyzed cord and the healthy one, which makes it easier to speak -- but a little harder to breathe Merati explained.

It’s very uncommon to have both cords weakened after viral infections, Merati said. “There are treatments, but they come with trade-offs,” he added. “We can make the voice stronger, but the aerobic capacity and the ability to exercise goes down. Or if we have a patient who says I want more air because I love mountain biking and I don’t care what I sound like, we can open up the gap.”

Angelina Jolie's surprising announcement that she'd had both breasts removed to reduce her risk of getting cancer has brought renewed attention to the controversial procedure.

Rates of women who are opting for preventive mastectomies have increased by an estimated 50 percent in recent years, experts say. And surveys show they are happy with the decision.

Splash News

Undated photo of Angelina Jolie, her mother, Marcheline Bertrand, and her brother, James. Bertrand died of cancer in 2007. She was 56.

But many doctors are puzzled because the operation doesn't carry a 100 percent guarantee, it's major surgery -- and women have other options, from a once-a-day pill to careful monitoring. Women can take tamoxifen or one of several newer drugs called aromatase inhibitors and reduce their risk by as much as 50 percent.

For Jolie, the chance to prevent cancer was worth losing her breasts, she wrote in the New York Times. 

Like many other women having the procedure, Jolie, who is 37 and a mother of six, says she did not want to live in dread of the cancer that killed her mother at age 56. “I decided to be proactive and to minimize the risk as much I could,” she wrote.

Since genetic tests for breast cancer risks have become available, the numbers of women choosing to be tested and then to have their breasts removed has shot up, says Dr. Todd Tuttle, chief of surgical oncology at the University of Minnesota.

Jolie said she had a mutation of the BRCA1 gene, which raises the risk of both breast and ovarian cancer. “My doctors estimated that I had an 87 percent risk of breast cancer and a 50 percent risk of ovarian cancer, although the risk is different in the case of each woman,” she wrote. She says she also plans to have her ovaries removed at some point.

In Jolie's case, her decision was "absolutely indicated," said Tuttle. At 37, Jolie is young to worry about breast cancer. But studies also show that the younger a woman is when she develops breast cancer, the more aggressive the disease is.

Other genes can raise or lower the risk that BRCA1 and BRCA2 mutations confer. And these mutations are rare. The U.S. Preventive Services Task Force recommends that only women with a strong family history even think about getting a BRCA genetic test –which is only 2 percent of U.S. women.

But why are so many women opting for surgery when survival rates for breast cancer are 93 percent if it’s caught at the earliest stages and 88 percent at stage 1?

“I have postulated that one of the downsides of breast cancer awareness is that there is a situation of hyperawareness. Women in the United States are just assuming they are going to get breast cancer,” Tuttle says. The actual rate is about 12 percent. About 1 in 8 U.S. women will develop breast cancer, and while 230,000 women were diagnosed with breast cancer last year, just under 40,000 died of it.

Dr. Sandra Swain, president of the American Society of Clinical Oncology, agrees that women shouldn't just assume they are at high risk. But she doesn't think there's any such thing as too much awareness.

"To me, you never can be too aware," says Swain, medical director of the Washington Cancer Institute at MedStar Washington Hospital Center. "I think people speaking out like Angelina Jolie are very good. She is very thoughtful about it." Jolie got genetic counseling and got an assessment of her own personal risk. "That's a good model," Swain said.

It’s hard to determine the precise number of women who are opting to have surgery for a medical condition they don't yet have. Private insurance companies have the best information, and there’s not an easy way to get it and compile a database.

Tuttle’s done a lot of research looking at how many women chose to have both breasts removed when cancer was found in one breast. Although the risk of developing cancer in the healthy breast is fairly low, many women choose to have both breasts removed when a tumor develops in one.

One study showed that women aged 55 and younger with a family history of breast cancer in both breasts – a high-risk group – had about a 16 percent risk of developing cancer in the second breast over the next 10 years. Older women would have an even lower risk. Yet the rates of prophylactic mastectomies among these women doubled between 1998 and 2005.

“It is pretty clear that the use of double mastectomy for women with cancer in one breast has exploded,” Tuttle told NBC News.

Another way to look at rates is to study women with a form of pre-cancer called lobular carcinoma in situ, or LCIS for short. LCIS does not always progress to cancer, but some women choose to have their breasts removed after a diagnosis, Tuttle says.

“Rates of prophylactic mastectomy for women with LCIS increased by 50 percent since the year 2000,” Tuttle said. He presented a study to the American Society of Breast Surgeons last week showing rates of women have preventive mastectomies after LCIS went from 12 percent in 2000 to 18 percent in 2009.

Jolie’s decision resonated with women like Lizzie Stark, 31, of Edison, N.J., who had a preventive double mastectomy two years ago after learning she had the BRCA1 gene. Her immediate response was empathy for the movie star – “This is a terrible decision to have to make” – and gratitude that Jolie chose to go public.

“I think it’ll make it easier, the more women who come out and talk about it,” said Stark.

Private insurers usually pay for both the removal and the reconstruction, including implants, if a doctor indicates the need. And the results are good if done by a good surgeon, studies show. Women usually feel good about their choice, also – surveys of women who have had double mastectomies show little regret.

But women may not realize just how serious the surgical procedure is, Tuttle says.

“I wonder if one of the reasons driving this trend is women underestimate the extent of this operation,” he said. “Prophylactic mastectomy with immediate reconstruction is a big operation. It can be five to six hours,” Tuttle says. “There can be complications and re-operations.”

And recovery can take a “good month”,  he added.  

“Prophylactic mastectomy is permanent and irreversible,” the National Cancer Institute cautions. “This surgery causes significant loss of sensation in the breast, which can have an impact on sexuality.”

Stark, who also had nipple-sparing reconstructive surgery, said she appreciated that Jolie made a point of saying that the surgery didn’t diminish her sexuality.

“I did feel like I lost my femininity,” Stark said. “Because it is a part of your body associated with femininity. I started wearing girlier clothes than I had before. I started wearing more makeup and plucking my eyebrows. But you don’t have to lose your femininity.”

Jolie explains the motivation behind her decision: control. "Life comes with many challenges. The ones that should not scare us are the ones we can take on and take control of," she writes.

Related:

• "I'm about to have a double mastectomy"
• New genetic clues found for breast cancer
• New study finds big batch of cancer genes

By Colleen Jenkins
Reuters

A South Carolina couple sued doctors and state social workers on Tuesday for subjecting a 16-month-old child born with both male and female genitalia to what they say was medically unnecessary and irreversible sex-assignment surgery while the toddler was in foster care. 

The state and federal lawsuits - believed by the couple's lawyers to be the first of their kind in the United States - argue that doctors should not have performed surgery to make the child's body appear to be female when they knew they could not predict how gender would develop.

The child, now 8, has shown strong signs of identifying as male and recently began living as a boy, according to Pam and Mark Crawford, who adopted him after the surgery.

The couple, a psychiatrist and stay-at-home dad, said they are taking legal action in the hopes of helping other children who face similar medical conditions.

"We feel very strongly that these decisions to permanently alter somebody's genitalia and their reproductive ability for no medical reason whatsoever is an abhorrent practice and can't be continued," Pam Crawford said in a phone interview.

"It is too late for our son," she added. "The damage has been done to him."

The lawsuit filed in state court in Columbia names the South Carolina Department of Social Services, Greenville Hospital System and Medical University of South Carolina as defendants.

Spokeswomen for the state department and the hospital system said they could not comment on the pending litigation, which alleges gross negligence and medical malpractice based on a lack of informed consent.

A spokeswoman for the Medical University of South Carolina - where the surgery took place - said the suit would be reviewed by the hospital's leadership and general counsel.

The child, identified only as M.C., was born in South Carolina in November 2004 and entered the state's foster care system in February 2005 after being removed from the custody of his biological parents.

Shortly after he was born, doctors noted that he had "ambiguous genitals" and both male and female reproductive organs, the lawsuits said.

Though doctors determined that he could be raised as either a boy or a girl, they opted for genital surgery that made the child's body look female - a decision the Crawfords say was premature because his dominant gender identity had not yet emerged.

"The surgery eliminated M.C.'s potential to procreate as a male and caused a significant and permanent impairment of sexual function," according to the filings in state court.

"The doctors knew that sex assignment surgeries on infants with conditions like M.C.'s poses a significant risk of imposing a gender that is ultimately rejected by the patient," the lawsuit says.

The federal lawsuit, filed in U.S. District Court in Charleston, South Carolina, says the surgery violated the child's constitutional rights.

Risks not fully explained
Sex-assignment surgeries on infants with intersex conditions, previously called hermaphroditism, have been performed since the 1950s, according to the Southern Poverty Law Center and Advocates for Informed Choice, which are helping represent the Crawfords and their child. 

The medical procedures often are performed without the risks being fully explained, the groups said.

"Doctors often assume they have to do surgery to make their bodies fit the stereotypes," said Anne Tamar-Mattis, executive director of Advocates for Informed Choice, a legal advocacy group for children born with variations of reproductive or sexual anatomy. In the case of the Crawfords' child, "the doctors knew from the beginning that there was a strong probability that this child would be a boy."

The Crawfords, who live in Columbia, said they initially raised M.C. as a girl after gaining custody when the toddler was about 20 months old.

But they said they soon noticed M.C. tended toward interests typically associated with males and preferred boys' clothing. As he grew older, he asked for his hair to be cut short like his father's and wanted to join a gymnastics class as a boy, they said.

"He's always been able to amuse himself with a toolkit," Mark Crawford said. "He's more likely than any of our other children to be climbing trees, wanting to ride bikes, flying model airplanes."

A couple of months ago, in consultation with their pediatrician, the Crawfords said their son made the transition to live as a boy and has been accepted as one by his friends.

But they added that the long-term consequences of the surgery are heartbreaking to try to explain to him.

"It's really tough because he's now asking the questions about how he can be like everybody else," Pam Crawford said.

By Trevor Stokes
Reuters

Bottling up emotions is thought to harm both mind and body, but a new study suggests that the opposite extreme may be no better.

In a study of thousands of heart attack patients, those who recalled having flown into a rage during the previous year were more than twice as likely to have had their heart attack within two hours of that episode, compared to other times during the year.

"There is transiently higher risk of having a heart attack following an outburst of anger," said study author Elizabeth Mostofsky, postdoctoral fellow with the Cardiovascular Epidemiology Research Unit at Harvard Medical School in Boston.

The greater the fury - including throwing objects and threatening others - the higher the risk, Mostofsky's team reports in The American Journal of Cardiology. The most intense outbursts were linked to a more than four-fold higher risk while milder bouts of anger were tied to less than twice the risk.

"The association is consistently stronger with increasing anger intensity; it's not just that any anger is going to increase your risk," Mostofsky told Reuters Health

The data came from a group of 3,886 patients who were part of a study between 1989 and 1996 to determine what brought on their heart attacks.

Within four days of having a myocardial infarction - the classic "heart attack" - participants were asked about a range of events in the preceding year, as well as about their diets, lifestyles, exercise habits and medication use.

A total of 1,484 participants reported having outbursts of anger in the previous year, 110 of whom had those episodes within two hours of the onset of their heart attacks.

Participants recalled their anger on a seven-point scale that ranged from irritation to a rage that caused people to lose control.

The researchers found that with each increment of anger intensity, the risk of heart attack in the next two hours rose. That risk was 1.7 times greater after feeling "moderately angry, so hassled it shows in your voice;" and 2.3 times greater after feeling "very tense, body tense, clenching fists or teeth" and 4.5 times greater after feeling "enraged! lost control, throwing objects, hurting yourself or others."

The most frequent causes of anger outbursts that participants recalled were family issues, conflicts at work and commuting.

Although the research cannot prove that the angry outbursts led to the heart attacks, the results "make sense," according to Dr. James O'Keefe Jr, a cardiologist at St. Luke's Hospital in Kansas City who wasn't involved in the research.

Anger is an emotion that releases the fight-or-flight-response chemicals epinephrine and norepinephrine, he said.

Those hormones raise our blood pressure, our pulse, constrict blood vessels, make blood platelets stickier (increasing the risk of blood clots), which O'Keefe says could be one way anger may be associated with increased heart risk.

"Contrary to the urban myth that it's best to express anger and get it out there, expressing anger takes a toll on your system and there's nothing really cathartic about it," O'Keefe told Reuters Health.

"(Anger) serves no purpose other than to corrode the short and long-term health of your heart and blood vessels," he said.

In the study, patients on blood pressure medications known as beta blockers had a reduced chance of having a heart attack following an angry outburst, Mostofsky's team notes in their report.

The authors say that finding suggests doctors might consider using those drugs preventively in people at risk of heart attack and prone to anger.

In discussing other possibilities for protecting people at risk, the researchers also write that during the 1990s when the data were collected, not enough study participants were on the newer statin drugs to determine their potential effects on heart attack risk.

Similarly, the number of participants who were on antidepressants was too low to tell whether they would have made a difference.

Regular exercise, Mostofsky and her colleagues write, has been shown to lower overall heart attack risk. Though they found no differences in the link between angry outbursts and short-term heart attack risk among regular exercisers in the study, they conclude that maintaining an active lifestyle couldn't hurt.

The study is part of a broader field of research looking at managing the effects of emotional states on cardiovascular systems, said Donald Edmondson, assistant professor of behavioral medicine at Columbia University Medical Center in New York, who studies heart attack survivors but was not involved in the new work.

"People prone to angry outbursts or more broadly, who are prone to anxiety, depression or other intense emotions should be aware that this is something that impacts their cardiovascular system," Edmondson told Reuters Health.

An unusual medical brawl erupted on Tuesday when the influential Institute of Medicine issued a report questioning the basis of years of advice for Americans to cut their salt intake in half.

The Institute, which advises the federal government on medical issues, concluded that the studies have answered the question poorly.  A panel of experts looked at studies showing the medical effects of eating too much salt, as well as at studies that have been used to suggest that some people may suffer form eating too little salt.

Its finding: There’s enough evidence to support advising Americans to keep sodium to 2,300 milligrams a day or less, but there’s not enough to support the current, lower target of 1,500 milligrams a day.

“There is evidence to lower excessive salt intake,” Dr. Brian Strom, a professor of medicine, epidemiology and biostatistics at the University of Pennsylvania, who chaired the panel, told reporters.

There is also good evidence that lowering intake to around 2,300 milligrams a day decreased the risk of heart disease,” Strom said. But he said that the evidence was absent to support recommendations of taking it as low as 1,500 milligrams a day. And, he said, there were some studies suggesting this could harm some people -- although those studies are also flawed.

This infuriated the American Heart Association.

“While the American Heart Association commends the IOM for taking on the challenging topic of sodium consumption, we disagree with key conclusions,” Heart Association CEO Nancy Brown said in a statement.

“The report is missing a critical component -- a comprehensive review of well-established evidence which links too much sodium to high blood pressure and heart disease.”

The Institute agrees that Americans eat far too much salt -- more than 3,400 milligrams a day on average. Most is “hidden” salt, in processed foods such as bread and cereal, restaurant meals and especially fast food.

And studies clearly show that salt raises blood pressure and that cutting salt can lower blood pressure. High blood pressure damages blood vessels and can lead to stroke, kidney failure and heart failure.

“We knew about all this data and it didn’t change our thinking,” said Dr. Elliott Antman of Brigham and Women’s Hospital and Harvard  Medical School . “We have to take the evidence that we have -- which is strong,” added Antman, a spokesman for the American Heart Association.

Several studies have suggested that about a quarter of Americans are salt-sensitive -- that their blood pressure is directly and immediately affected by how much salt they eat. But this doesn’t mean that the other 75 percent can pile on the salt with impunity, Antman said.

“About 90 percent of the population will ultimately develop hypertension as they age,” he said. “Part of this is almost certainly from years of eating too much salt, he added. Eating too much salt can also affect kidney function, he said.

“An aggressive reduction in sodium is the way to go,” he said.

Strom would not be drawn into an argument. “What we are saying is the available data is not consistent on outcomes,” he said. “We are not saying that one shouldn’t be lowering excessive salt intake in the general population,” he added.  “There is simply a lack of data showing it is beneficial.”

And, he said, it is not up to the panel to set a target. “It’s not that we are against (a target intake of) 1,500 milligrams,” he said. “The data are not consistent.”

The trouble with trying to measure salt intake is that it’s very hard to separate out salt from the rest of the  ingredients in food, said Antman.

A truly scientifically rigorous study would randomly assign people to eat diffferent amounts of salt daily and watch to see what kind of diseases they develop -- something that would be difficult to set up, police and also difficult to defend, ethically.

Antman says most Americans get their salt from prepared foods and from restaurants, not from at-home, scratch cooking. What’s needed, he said, is more encouragement for restaurants and food companies to lower sodium content of food.

The food industry argues that Americans have a taste for salt and they won’t buy products that are salt-free.  Antman agrees that it’s hard.  “We are used to a high sodium content in our food,” he said.

“But the data say that in just two to three weeks you can train person’s taste buds to become accustomed to a lower sodium content in food.”

The Center for Science in the Public Interest piled on, too.

"What the committee failed to emphasize is that most Americans are deep in the red zone, consuming 3,500 to 4,000 milligrams of sodium a day,' the group, which publicizes calorie counts and other food risks, said in a statement.

"It's clear that those excessive levels increase the risk of high blood pressure, heart attacks, and strokes. Whether we aim for 2,300 or 1,500 milligrams a day is irrelevant until we move down out of the red zone. At restaurants, you can get roughly 2,000 milligrams of sodium from just one burrito, a single-serve pizza, or an order of kung pao chicken, and at least 1,000 milligrams from a typical sandwich or burger."

The Centers for Disease Control and Prevention has a widget on salt intake here.

Related:

• Too much salt may trigger autoimmune disease
• US advises Americans to eat less salt
• Most teens well down the road to heart disease

The Obama administration filed its expected appeal on Monday of a judge's order to make the so-called "morning-after pill" available to anyone, without a prescription.

The federal government asked the 2nd U.S. Circuit Court of Appeals in New York to postpone federal judge Edward Korman’s ruling against the Health and Human Services Department’s requirement that anyone under 17 get a prescription for the emergency contraception.

Korman refused on Friday to stay his ruling while the appeals court considered the appeal, calling the government’s legal attempts “frivolous” and saying they were done in bad faith.

Women’s health groups had sued against the HHS decision, which had overruled the Food and Drug Administration’s original approval of Plan B birth control pills for women and girls of all ages. HHS Secretary Kathleen Sebelius had said she wasn’t comfortable seeing the pills freely available to girls as young as 11.

The FDA, which had protected against Sebelius’s decision, last month approved another drug application to make one brand available over the counter to girls as young as 15, but still requiring proof of age.

Korman railed against both decisions and has accused the government of simply trying to delay the inevitable.

For now, things are a bit confusing. The appeal, technically filed by the Food and Drug Administration, asks the appeals court to stop Korman's ruling from taking effect until the case is settled.

Teva Pharmaceutical’s single-pill option, called Plan B One-Step, is available with ID to anyone 15 and older. Anyone younger needs a prescription to get it.

Other versions of the medication are available to anyone 17 and older with proof of age, and to anyone younger with a prescription.

"You have to show an ID either way," said Judy Waxman of the National Women's Law Center. Waxman  predicts the appeals court will stay Korman's decision until a hearing can be scheduled on the appeal. "This could take months again until we get the Second Circuit decision," Waxman said in a telephone interview.

Related:

Judge refuses to delay Plan B ruling, has choice words for HHS

Judge: Make 'morning-after pill' available to all girls without prescription

West Nile virus killed 286 people in the United States last year, making it the deadliest year yet for the virus, the federal government reported on Monday.

Texas was especially hard hit by the virus, which is carried by mosquitoes and which only arrived in the United States in 1999, the Centers for Disease Control and Prevention reports.

“A total of 5,674 cases of West Nile virus disease in people, including 286 deaths, were reported to CDC from 48 states (excluding Alaska and Hawaii),” the CDC said in a statement.

West Nile virus is widespread in Africa, Asia and parts of Europe. It causes fever and aches and usually isn’t serious.  But in some people it can spread to the brain or spinal cord, killing them or causing paralysis.

No one’s sure precisely how West Nile arrived in North America, but it was first reported in New York in 1999. It’s now been reported in all 48 contiguous states, as well as parts of Canada and Mexico and all the way to Argentina.

The CDC's Dr. Lyle Petersen says it's impossible to know what West Nile will do this summer. "It is very hard to predict," he said in a telephone interview. "I can't tell you what the weather is going to be like this summer, for example." The virus is driven by weather; it's worse during hot, wet summers in temperate climates.

"What last summer's outbreak tells us is that West Nile is not going to go away," Petersen said. "Most places in the United States are at risk of having outbreaks."

The virus has a life cycle that takes it from mosquitoes to birds and back into mosquitoes that bite people. Its severity varies from year to year. In some years, only a few cases are reported, and in others, like 2012, it infects many people.

In 2011, CDC reported 712 cases of West Nile virus, and 43 deaths. The worst previous year was 2003, when 9,862 cases were reported with 264 deaths. Only severe cases are reported to CDC -- health experts say most people who are infected don’t even know it. People over 50 and people with underlying illnesses are the most vulnerable.

“Last summer’s outbreak likely resulted from many factors, including higher-than-normal temperatures that influenced mosquito and bird abundance, the replication of the virus in its host mosquitoes, and interactions of birds and mosquitoes in hard-hit areas,” the CDC said in a statement.

“Because the factors that lead to West Nile virus disease outbreaks are complex, CDC cannot predict where and when they will occur."

There’s no vaccine against West Nile virus for people, but there is one for horses. There’s also no specific antiviral drug that can help infected people -- those who are seriously ill get what’s called supportive treatment in the hospital.

The CDC recommends that people avoid being bitten by mosquitoes, which can carry other diseases, also, from Eastern equine encephalitis to, in very rare cases, dengue fever. Standing water, even in extremely small containers, can breed mosquitoes. People should wear long sleeves and use insect repellent that contains DEET, picaridin, IR3535, and para-menthane-diol (PMD), CDC advises.

Petersen says scientists don't quite understand why, but West Nile tends to cause more human outbreaks in temperate climates than in tropical zones. Female mosquitoes carry the infection over from one summer to another, he said. "Infected female mosquitoes find a warm place to live and they just hang out all winter," he said. They often overwinter in sewers or basements, he said.

"These infected mosquitoes come out in the spring and then they look for a blood meal and they bite birds and infect birds. During spring and summer you get more and more infected mosquitoes and birds," Petersen added.

"By the middle of summer there are so many infected mosquitoes that it starts to present a human infection risk."

And because the virus can live in both mosquitoes and birds, it would be much more difficult to eradicate than malaria, which mostly infects people, Petersen said. The CDC helped eradicate malaria in the United States in 1951.

Related:

• Is spraying for West Nile safe?
• Don't expect hurricane effect from West Nile