Trisomy 18 and 13: More Children Like Bella Santorum Survive

Bella Santorum, the youngest daughter of Republican presidential hopeful Rick Santorum, was hospitalized on Friday, the second time this year she has needed such care.
Bella, age 3, suffers from Trisomy 18, a rare genetic disorder that is often considered fatal. Her survival has defied the odds. About 90 percent of children born with the disorder die in their first year of life. Santorum has discussed his daughter's condition many times on the campaign trail, describing her survival as "miraculous."
But according to a new study, a significant number of children with the condition are actually surviving to older ages, though it takes a lot of medical care to keep them going.
More than a third of hospitalizations for Trisomy 18 and the closely related Trisomy 13 over the past 15 years were for children more than a year old, the study said. In more than 10 percent of the cases, the child was beyond age 8.
Study author Dr. Chris Feudtner said the analysis, published today in the journal Pediatrics, shows that the conventional thinking that the disease is lethal is not totally accurate.

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"The norm is still great difficulty with survivorship for most of the children. But to say that is true for most does not mean that is true for all," he said.
Trisomy 18 and 13 occur in children who have three chromosomes for a particular gene when there should be only two. The conditions also are closely related to Trisomy 21, the scientific term for Down syndrome.
The conditions cause symptoms like clenched hands, low birth weight, mental deficiencies, small head and jaw and an unusually shaped chest, according to the National Institutes of Health. Diseases like pneumonia that are typically not life-threatening in other children can be for those with Trisomy 13 and 18.
In January, Bella Santorum was hospitalized with pneumonia, but recovered.
For the current study, Feudtner and his colleagues analyzed data on hospitalizations for children with Trisomy 18 and Trisomy 13. As many as 1,600 hospitalizations in the U.S. during the last 15 years were for patients with Trisomy 18 and up to 900 hospitalizations were for patients with Trisomy 13. Those patients underwent 2,765 major medical procedures, for everything from cleft palate surgery to heart surgery to repairs to the gastrointestinal tract.
Dr. Robert Marion, director of the Center for Congenital Disorders at The Children's Hospital at Montefiore Medical Center, said the study reflects a shift in philosophy on how to treat children who have such severe genetic disorders.
As recently as five to ten years ago, many doctors believed it would be wise to consider ending the extensive medical intervention those children received to prolong life. Maybe it would be better for nature to take its course.
"Thankfully, this has changed in recent years. Not only do some of these children survive, they survive with a pretty good quality of life," Marion said. "In children who are doing reasonably well, we will perform surgical procedures or provide more aggressive feeding therapy in order to give the kids a chance to make it."
Still, children who survive beyond age 1, like Bella Santorum, are the exception rather than the rule.

Santorum Wins Louisiana Primary

MANDEVILLE, La.—Rick Santorum won the Louisiana Republican presidential primary on Saturday, as voters in this conservative, Southern state decided they weren't ready to coalesce around front-runner Mitt Romney.
Exit polls showed Mr. Santorum winning with his most dominant performance yet this year among conservatives and blue-collar voters and getting robust support from nearly all types of people, according to the Associated Press.

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Rick Santorum greeted supporter at a rally at in Sheboygan, Wis.

Mr. Santorum was grabbing the votes of nearly half of conservatives, and just more than half of those without college degrees—a measure of blue-collar voters.
With 100% of the precincts reporting, Mr. Santorum beat Mr. Romney, 49% to 27%.
But the former Pennsylvania senator's victory will give him only a modest boost to his efforts to blunt Mr. Romney's push toward the nomination. Only 20 delegates were at stake Saturday, and they may be divided among several candidates. Mr. Romney holds a lead of about 300 delegates over Mr. Santorum, according to the AP.
An ebullient Mr. Santorum, who had moved his campaign to Wisconsin on Saturday, told an enthusiastic election-night crowd in Green Bay, Wis., that he will continue pressing for the nomination.
"The people of Louisiana sent a loud and clear message: This race is long and far from over. And the people of Wisconsin, I just say to you: On Wisconsin, let's get it done."
Mr. Santorum, who had visited a bowling alley earlier in the day, was eager to share his success there, too.
"I don't want to brag, but I did bowl three strikes in a row. I just wanted to say that. This has been a pretty strong day on the campaign here in Wisconsin," he said. Wisconsin holds the next big nominating contest, on April 3, and recent polls have shown Mr. Romney in the lead.

Rick Santorum thanked Louisiana voters during a news conference in Green Bay, Wisconsin after he won the Republican primary in the Southern state, beating front-runner Mitt Romney. (Video: NewsCore/Photo: AP)

The Louisiana win comes as an increasing number of Republican leaders say it is time for the party to unify behind Mr. Romney and turn to the challenge of defeating President Barack Obama this fall.
Earlier this week, former Florida Gov. Jeb Bush, a leading conservative, endorsed Mr. Romney. Sen. Jim De Mint (R., S.C.), a leading figure among tea-party supporters, said he was "excited about the possibility" of the former Massachusetts governor "possibly" being the nominee. But Louisiana voters disagreed.

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"I definitely voted for Rick Santorum," said Dennis Calkins, a 62-year-old retiree who cast his ballot at Mandeville High School. "I am pretty conservative, and he just seemed down-to-earth and no-nonsense."
Mr. Calkins said he liked how Mr. Santorum responded to informal questions on a television show months ago.
"I liked every answer that he gave," Mr. Calkins said. He said Mr. Santorum's Christianity also influenced his vote. "It's not so much that he's holy moly and religious, but it gives him a basis for morality and his sense of right and wrong."
Loretta Owens, 62, who attended a Santorum rally Friday in West Monroe, in the northern part of the state, said she had reservations about Mr. Romney. "There's something about Romney that I just can't grasp," she said.
Mr. Santorum had aggressively courted conservative voters in the northern part of Louisiana, the same area that helped Mike Huckabee claim victory in the 2008 GOP primary. In his pitch to Republican voters there, Mr. Santorum said he would stand up to big government in Washington, stand firm against abortion rights, and would live up to his campaign promises.
Mr. Santorum began labeling Mr. Romney the "Etch A Sketch candidate," whose positions would change like the draw-and-erase toy. The line came after a Romney aide said that the former governor would make a pivot at the time of the general election, "almost like an Etch A Sketch."
But it was during this leg of the campaign that Mr. Santorum made what rival Newt Gingrich described as his "biggest single mistake." Mr. Santorum said that if a candidate is "going to be a little different than the person in there," then "we ought to stick with what we have." His opponents said it was outrageous that Mr. Santorum would consider backing Mr. Obama over a GOP nominee.
Mr. Santorum later said he had been inarticulate, and that it was "preposterous" to suggest that he would pick Mr. Obama over a Republican. He said he would support the eventual Republican nominee, a reminder that Mr. Romney has been racking up more delegates than Mr. Santorum and taken a hard-to-overcome lead.
Along with Wisconsin on April 3, Maryland and the District of Columbia hold primaries. After that, the race heads to the Northeast, including the delegate-rich states of Pennsylvania and New York.

Dick Cheney recovering at hospital after heart transplant

Former Vice President Dick Cheney was recovering Saturday at a Virginia hospital after receiving a heart transplant, his office said.

Cheney was in the Intensive Care Unit of Inova Fairfax Hospital in Falls Church, his office said.

Cheney, 71, who served as vice president in the George W. Bush administration, has had a long history of heart trouble and has been on the cardiac transplant list for more than 20 months.

"Although the former Vice President and his family do not know the identity of the donor, they will be forever grateful for this lifesaving gift," aide Kara Ahern said in a written statement that was authenticated by several close associates of the former vice president.

More than 3,100 Americans currently are on the national waiting list for a heart transplant. Just over 2,300 heart transplants were performed last year, according to the United Network for Organ Sharing. And 330 people died while waiting.

According to UNOS, 332 people over age 65 received a heart transplant last year. The majority of transplants occur in 50- to 64-year-olds.

The odds of survival are good. More than 70 percent of heart transplant recipients live at least five years, although survival is a bit lower for people over age 65.

NBC's Robert Bazell explains the process of receiving a heart transplant amid news that Former Vice President Dick Cheney has received a new heart.

Cheney suffered a heart attack in 2010, his fifth since the age of 37. That year, he had surgery to have a small pump installed to help his heart keep working. It was one of the few steps left, short of a transplant, to stay alive in the face of what he acknowledged was "increasing congestive heart failure."

The pump, called a left ventricular assist device, is mainly used for short periods to buy time for potential transplant candidates awaiting a donor organ. The fact that doctors resorted to it illustrated the perilous condition he was in.

"I've gotten used to the various contraptions that are always with me, and I'm working and traveling, I've hunted a time or two, and I have some fishing planned," Cheney wrote in his memoir released last year.

In July 2007, he had had a minor surgical procedure to replace a device that monitored his heartbeat. Nearly 20 years earlier, in 1988, Cheney had had quadruple bypass surgery, and had two artery-clearing angioplasties and the operation to implant the device.

In 2005, Cheney had six hours of surgery on his legs to repair a kind of aneurysm, and in March 2007, doctors discovered deep venous thrombosis in his left lower leg. An ultrasound a month later showed the clot was getting smaller.

In January 2011, Cheney said he was getting by on a battery-powered heart pump, which made it "awkward to walk around." He also said he hasn't made a decision yet on a transplant, but that "the technology is getting better and better."

Cheney said then that he'd "have to make a decision at some point whether I want to go for a transplant."

Like 5 million other Americans, Cheney had congestive heart failure, meaning his heart had become too weakened to pump properly. That can happen for a variety of reasons, but Cheney's was due to cumulative damage from five heart attacks that he had suffered since age 37.

Heart failure kills 57,000 Americans a year and contributes to many more deaths.

Cheney served as Bush's vice president for eight years, from 2001 until 2009. Cheney was a lightning rod for criticism during Bush's presidency, accused by opponents of often advocating a belligerent U.S. stance in world affairs during wars in Iraq and Afghanistan