Experts say a Maryland man who died last month of rabies might have been spared under transplant recommendations that hadn't yet been published when he got a kidney from an infected donor in 2011.

The guidance from the federally funded United Network for Organ Sharing came out in June, nine months after organs from the Florida donor went to four patients. The guidelines urge caution when considering donors with encephalitis, a brain inflammation that is one symptom of rabies. The Florida donor suffered from the condition.

The only other known U.S. case of rabies transmission through a transplant occurred in 2004 in Texas. Four organ recipients died, and the case helped stir the creation of a UNOS committee dedicated to reducing disease transmission through transplants. The Disease Transmission Advisory Committee was formed in 2006 as part of a government agreement with the private group, based in Richmond, Va.

The committee says it took so long to publish the encephalitis guidelines partly because it had to gather and analyze enough data about rare transmission events to provide a solid basis for its recommendations.

The committee also decided to address more common diseases first.

In 2010, it issued guidance on influenza, suggesting that lungs and intestines from donors infected with the H1N1 virus not be recovered for transplant. In 2011, the panel published guidance on the cancer-causing HTLV-1 virus, and last November, recommendations on tuberculosis. The group now is working on guidance for West Nile virus.

The encephalitis document urges organ banks and transplant surgeons to be cautious in considering organs from donors whose symptoms suggest a central nervous system infection, such as rabies. It urges "extreme caution" when the encephalitis appears to be from a virus. The document lists the rabies virus as one possible cause of encephalitis.

When the donor — 20-year-old William Edward Small — died in September 2011, doctors at Sacred Hearth Heath System in Pensacola, Fla., thought his symptoms were caused by a food-borne toxin, ciguatera, carried in saltwater fish, said Kathy Giery, director of LifeQuest Organ Recovery Services, which approved his organs for transplant.

She said nobody suspected the donor had rabies until after the Maryland recipient died. If they had, the organs wouldn't have been offered, she said.

"The guidelines from 2012 were different from what was in place in 2011, when this took place, so for our cases today and going forward, I think everyone would be looking at this through a different lens," Giery said.

Dr. Michael Green, a University of Pittsburgh pediatric surgeon and chairman of the Disease Transmission Advisory Committee, said a concern for rabies in potential donors would call for extreme caution. But he said every transplant carries risk.

"In every instance, the transplant team must weigh the rare risk of donor disease transmission against the possibly greater risk that a candidate will die without a transplant opportunity," Green wrote in an email.

Compared with other diseases, rabies is rare. It kills just a handful of U.S. residents a year. Usually transmitted by a wild animal bite, it's almost always fatal once symptoms appear. But the disease can be stopped if a bitten person gets treatment, including a series of vaccinations, soon after exposure. The people in Florida, Georgia and Illinois who also received organs from Small have shown no symptoms and are getting the vaccines.

Thirty-two other people in five states who had close contact with the recipients or donor also have been urged to get the vaccine, according to the Centers for Disease Control and Prevention.

Committee member Dr. Timothy Pruett, surgical chief of the University of Minnesota transplant programs, said deaths from transmitted diseases are horrible events — but part and parcel of the organ donation system.

"The risk of dying without an organ still is so much greater than the risk of dying with a transmitted disease, but I don't want to be cavalier about it — it's something no one wants to see," he said.

The eight years it took for the rabies guidance seems unreasonable to Jennifer Hightower of Gilmer, Texas, whose 18-year-old son Joshua was among those killed by rabies-infected transplants in 2004.

"I think it took way too long," she said. "I believe any time there's signs of encephalitis or any kind of anything wrong with the brain that they can't point to, they shouldn't use them.

Related:

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Woman says rabies-infected donor was her child's father

Associated Press
Miami doctors say they believe a five-organ transplant patient is the first to deliver a baby, making her the first reported case in the world. 

Fatema Al Ansari was 19 and living in Qatar when she was diagnosed with a blood clot in a major vein to the intestine. In 2007, she underwent surgery at Jackson Memorial hospital in Miami and was given a new liver, pancreas, stomach and small and large intestine. Five years later, she gave birth to a girl.

Al Ansari faced some complications during pregnancy, but her doctors say she is capable of having more children.

Al Ansari says it's "the best feeling in the world" to be a mother. Her doctors add there are no reported cases of a five-organ transplant patient in the world giving birth. 

Northwestern Memorial Hospital

Cera Fearing, 21, far right, donated her kidney last June to her brother, Ray Fearing, 27, center. When it started to fail, doctors re-implanted the organ into Erwin Gomez, 67. The kidney is working just fine, the Chicago-area patients say.

A single kidney that was transplanted twice in two weeks is working fine after what appears to be the first-ever case of doctors salvaging a hand-me-down organ after it started to fail.

Erwin Gomez, a 67-year-old Chicago-area surgeon and father of five, is the third and, hopefully, final owner of the kidney, which was transplanted last June 16 -- and again on June 30 -- at Northwestern Memorial Hospital in Chicago.

It originally came from Cera Fearing, a 21-year-old day care worker from Elk Grove, Ill., who donated the organ in hopes of helping her big brother.

“The whole point of giving it was to give him a new life,” she said.

Instead, Ray Fearing, 27, of Arlington Heights, learned within days of receiving Cera’s kidney that the organ was failing, attacked by a reoccurence of his longtime disorder, focal segmental glomerulosclerosis, or FSGS, a common kidney disease in which scar tissue develops on the part of the kidney that filters waste from the blood.

Dr. Lorenzo Gallon, the transplant expert who treated Fearing, said he just couldn't stand to let a good organ go to waste, especially in a nation where nearly 92,000 people are waiting for a kidney transplant.

"This kind of like haunted me," said Gallon, who is medical director of the hospital's kidney transplant program and associate professor of medicine and surgery at the Northwestern University Feinberg School of Medicine. "I thought, I just gotta do something so at least something good can come out of this."

Gallon had warned Fearing that there was perhaps a 50-50 chance that the kidney could fail because transplantation doesn't always stop FSGS. In addition, a reoccurence of the disease could threaten Fearing's life. When Gallon saw signs that it was happening, he consulted with his colleagues and with the hospital ethics board to discuss reusing the kidney in another patient.

"The kidney ... was still a relatively healthy, viable organ that could be transplanted into someone else without FSGS," said Gallon, whose feat is chronicled in a letter in the latest issue of the New England Journal of Medicine.

At first, the news was disappointing for both Ray Fearing and his sister.  

Video: Electrician sparks kidney donations

“When I found out it was going to be taken out of my brother, I was a little upset,” Cera Fearing said. “I figured it was going to go into the trash.”

Doctors offered Ray Fearing three options: Keep the new organ and hope for the best, remove the kidney and discard it, or donate it to someone else.

For Fearing, who was diagnosed with FSGS at age 15 and had spent 10 years hoping for a kidney, the choice was obvious.

“Why would I just throw out a kidney?” he said. “I know exactly what it’s like to want it so bad.”

That’s where Dr. Erwin Gomez came in. He’d been on the kidney transplant waiting list for about four months, after diabetes and poorly controlled high blood pressure left him on the verge of kidney failure.

“Initially, I was hesitant,” said Gomez, who worried about the condition of an organ that had already been transplanted once.

But when doctors told him the FSGS now affecting the transplanted kidney was reversible, and he would ultimately wind up with a healthy organ from a living, 21-year-old donor -- all without the typical five-year wait -- he decided to go ahead. Within days, the new kidney was functioning normally.

“I consider myself very blessed, with a well-matched kidney,” Gomez said.

Fellow kidney transplant specialists consider Erwin blessed as well. Dr. Matthew Cooper, director of the kidney transplant program at the University of Maryland, had never heard of a failing kidney being removed from an FSGS patient and re-implanted in another person.

“It’s pretty amazing stuff,” said Cooper, who chairs the National Kidney Foundation's "End the Wait Committee." “It’s very difficult once that kidney is transplanted to think about re-transplanting it.”

The trio that shared the kidney -- Cera Fearing, Ray Fearing and Gomez -- met for the first time Wednesday in an emotional encounter at the hospital.

Ray Fearing is now back on dialysis, with possible plans to seek another kidney transplant in a couple of years. He says he’s doing fine, and has recently discovered that he can travel and obtain dialysis in different locations across the country. 

Gallon, who expressed affection and admiration for Fearing and his family, says it may be possible for the young man to have another transplant in the future, and there's a chance that the organ will function properly. For other patients awaiting kidney transplants, he says the success of the secondhand implant could well spur new research into FSGS and new thinking about what to do when an organ fails.

"Instead of tossing them out, you'll reuse them," he said. "It opens the window to using organs that we would throw away." 

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Jackson Health System

Angela's father Petrit Bushi, Angela and her mother Valbona, smile after the 6-year-old girl received a new liver, two kidneys and pancreas.

Valbona Bushi, 28, knew even before the doctors did: Her daughter Angela’s liver was failing, just as Angela’s younger sister’s had.

At first, emergency room doctors dismissed the kindergartener’s flu-like symptoms as nothing serious. But the next day the 6-year-old's abdomen was slightly swollen. Bushi, whose 18-month-old daughter Alicia had died of liver failure just a few months earlier, rushed Angela back to the hospital near their home in Jacksonville, Fla. After tests revealed the little girl needed a liver transplant and she was referred to Holtz Children’s Hospital, affiliated with the University of Miami Miller School of Medicine.

A new liver would save Angela from imminent death, but doctors wanted to do more -- to try to actually extend her life. Angela, whose parents had moved to Jacksonville from Albania seven years ago, had been newly diagnosed with a rare, fatal genetic disorder called Wolcott-Rallison Syndrome. Only about 60 cases of the disorder -- which causes infant-onset diabetes and liver failure, as well as bone fractures, intellectual impairment and frequent infections -- have been reported. Only one of those children lived into young adulthood. It had killed Angela’s younger sister and damaged Angela's organs.

On December 29, in an unprecedented multiple-organ transplant on a child, Angela received a liver, two kidneys and a pancreas, doctors said.

 The operation -- the first time a transplant has been used to treat the disorder --  “might offer some hope for these children with this rare syndrome, that life can be prolonged, hopefully in a very meaningful way,” Dr. Andreas Tzakis, transplant surgeon, told msnbc.com Thursday. Tzakis is the chief of the liver and digestive tract program at Jackson Memorial Hospital.

A viral infection, the kind most kids easily bounce back from, caused Angela's liver failure, said Dr. Olaf Bodamer, director of the pediatric genetics program at Holtz Children’s Hospital. In children with Wolcott-Rallison, a protein doesn’t function normally. When patients contract a virus, their cells are unable to clear the resulting waste products, Bodamer explained. “Theoretically, any organ could be affected,” he says, but the liver, pancreas and kidneys are most vulnerable.

Angelad was diagnosed with Type 1 diabetes as as a baby, but, since her transplant, is no longer diabetic.

Her post-operative course “was tougher than expected,” says Tzakis. But she recovered and was discharged from the hospital on Feb. 23.

Now, Angela can’t wait to return to kindergarten which she had attended for only two weeks before becoming ill, her mother said.

“Every day she says, ‘I miss my school,’” Bushi says. Angela’s return to the germy world of elementary school will have to wait until she’s more stable, but Tzakis has “guarded optimism” that the transplant operation will halt or slow the normal course of her disorder.

“They saved my daughter’s life,” Angela’s mother Valbona told msnbc.com.

Three-year-old Amelia Rivera has a rare, very serious genetic disease known as Wolf-Hirschhorn Syndrome that can cause mental impairment, epileptic-like seizures and kidney failure. In the future, it's very likely she’ll need a transplant.

But, according to her parents, Amelia's mental disabilities are the reason she's being denied  the kidney transplant. Her parents told the Associated Press Wednesday that the decision is being determined by a doctor with The Children's Hospital of Philadelphia.

AP

The parents of 3-year-old Amelia Rivera, claim she is being denied a kidney transplant because of her mental disabilities.

"It's one doctor who's never seen us who is making this call," the father, Joe Rivera, told the Associated Press.

In a recent blog post written by her mother, Chrissy Rivera says they were told by her doctor and social worker that Amelia would not be a candidate for a transplant because of her mental disability, not even if a family member donates a kidney.

In the blog post, Rivera recounts this exchange with the doctor when she heard the news:

“So you mean to tell me that as a doctor, you are not recommending the transplant, and when her kidneys fail in six months to a year, you want me to let her die because she is mentally retarded? There is no other medical reason for her not to have this transplant other than she is MENTALLY RETARDED!”

“Yes, [said the doctor]. This is hard for me, you know.”

The blog post sparked an online firestorm -- including more than 15,000 signatures on a petition demanding that the decision be reconsidered by the Children’s Hospital of Philadelphia, an affiliate of the University of Pennsylvania where I work. 

In response to the uproar, the child's parents are planning to meet with hospital officials next week, according to AP.

The issue of disability and access to a life-saving transplant merits serious reflection.

Whether the kidney comes from a cadaver donor or a living one, transplant teams always think about a set of medical facts in deciding whether to transplant anyone. Is the person able to go on kidney dialysis? Is she healthy enough to survive surgery? Does the patient have a donor who closely matches her blood and tissue type increasing the chance that the transplant will work? There is nothing special about the presence or absence of mental disability with respect to these questions. But morally, things get a little stickier.

Those being considered for a transplant must be able to comply with what is required after a transplant — taking a lot of medicines and watching out for early signs of rejection of the transplanted kidney. This means that those with severe mental impairment need willing, round-the-clock helpers so that the transplant has a reasonable chance of succeeding.

Transplant teams also think about the chance for long-term survival, especially when a scarce cadaver kidney is involved. Some severe mental disabilities are linked to genetic problems that create other daunting physical challenges -- bad hearts, severe diabetes, malformed organs, etc., which can drastically shorten lifespan.

Because of that, some transplant programs don't consider operating on those who are facing significantly shortened lives on the grounds that the need for transplants among kids is huge and the scarce supply of cadaver kidneys should be used to save the most lives and the most years of life.

It’s even more complicated when the patient is a very disabled person who resides in a care facility. A child who is immunosuppressed, whose bodily defenses are knocked out to permit the transplant to work, will be put at huge risk of getting a deadly infection if she lives in an institution.

Each transplant case involving a disabled person has to be looked at individually.  All that said, children with intellectual disabilities do not appear on transplant waiting lists with the frequency that should be expected.  And in 1990 Congress passed the Americans with Disabilities Act  which prohibits discriminating against patients solely on the basis of a disability.

There are reasons why anyone with an intellectual or physical disability might not be considered a good candidate for a transplant.  But those reasons, to be ethical, have to be linked to the chance of making the transplant succeed. Otherwise they are not reasons, they are only biases.

What do you think? Discuss this column on Facebook. 

The Star-Ledger

Organ transplant experts are worried that proposed new federal health guidelines will limit the number of available donors and recipients willing to accept organs newly classified as risky.

If you've had sex with two or more partners in the past year, you may be considered a risky organ donor, at least according to proposed new federal health guidelines that have drawn sharp protests from transplant experts who say they're far too broad. 

“With the new guidelines, every college student in America will be high risk,” said Dr. Harry Dorn-Arias, a transplant surgeon at the University of Virginia. “Right now, it's probably a prostitute or a guy with a needle in his arm. Next time, it will be just a young guy."

Under the new policy proposed this fall by the Centers for Disease Control and Prevention, deceased and living donors who were not monogamous in the previous 12 months would be considered at increased risk of transmitting HIV, hepatitis B and hepatitis C -- even if they had no other risk factors

CDC officials say the proposed guidelines are aimed at making the organ supply safer and preventing accidental transmission of life-threatening infections. The policies wouldn’t absolutely ban anyone from donating, especially in an exceptional or life-saving situation, but they would call for more scrutiny and testing.

“It’s geared for the patient so the patient knows as much as they can about the organ being transplanted in them,” said Dr. Matthew J. Kuehnert, director of the CDC’s office of Blood, Organ and Other Tissue Safety.

But transplant experts are outraged because they say the proposal arbitrarily focuses on monogamy and could limit both the number of available donors and the number of recipients willing to accept organs newly classified as risky.

They worry that potential living donors may balk at donating if they know their sexual history alone could raise questions about their suitability, particularly if the situation involved a family member.

“If you were going to give your organ to your mom or dad or sister, you’re going to be ashamed of that,” said Dorn-Arias. “You’re either going to say no, or you’re going to lie.”

The proposed policy could also require families of deceased donors to answer uncomfortable questions -- ones they may not even know the answers to -- about the specific sexual behaviors of their loved ones.

“It’s probably going to triple what we consider high risk at this point,” said Tracy Giacoma, transplant administrator at the University of Kansas Hospital. “It may scare patients off from taking these organs. More patients may die because they don’t take these organs.”

More than 28,000 organs are transplanted each year, but more than 112,000 people are on organ waiting lists, according to figures from the Organ Procurement and Transplantation Network.  

The guidelines could affect a wide swath of potential donors, particularly younger people. About a quarter of women and nearly 30 percent of men ages 20 to 24 said they had two or more sexual partners in the past 12 months, according to a 2006-2008 report by the National Center for Health Statistics.

When tragic deaths occur, those are precisely the people who should donate their organs, if possible, Giacoma said.

"If you have a [donor] that's 19 years old and he had multiple partners, we'll have to tell the recipient, this is a high-risk organ," she said.

The sexual partner tally is only one of several new factors that could tag a potential donor as being at increased risk of infection. It’s part of a larger set of guidelines that would update 1994 Public Health Service policies for preventing transmission of HIV through human tissue and organs.

"Our priority here is patient safety," said Kuehnert, who noted that the guidelines describe "increased risk," not "high risk," of infection. "[Patients] should know if they're getting an organ at elevated risk."

The 1994 guidlines exclude certain groups as donors, including men who have had sex with other men within the past five years, people who've used IV drugs or exchanged sex for money or drugs in the past five years, hemophiliacs, those exposed to HIV, and people who've had sex with anyone in those categories. They also limit people who've been incarcerated.

The new plan calls for the first-ever guidelines for testing living donors and it adds hepatitis B and hepatitis C to the list of must-test viruses, along with HIV, Kuehnert said.  As it stands now, only HIV is included in the guidelines, though most organ transplant centers do test for a range of other potential diseases. 

The proposal also calls for use of the most sensitive tests available to detect infection and for shorter testing windows to avoid transmitting infections, which occurs in an estimated 1 percent of transplant cases and has been fatal, Kuehnert said.

Between 2007 and 2010, the CDC participated in more than 200 investigations of suspect unexpected transmission of infections including HIV and hepatitis B and C, with dozens of cases confirmed, Kuehnert added.

The risk of infection from organs may be rare, but it's real. Helen Boucher's husband, George, 54, of Pawtucket, R.I., died in 2005 after receiving a kidney tainted with a rare infection traced back to a virus from the donor's pet hamster. The new guidelines wouldn't have helped detect the Lymphocytic Choriomeningitis virus -- known as LCMV -- but Helen Boucher, now 61, said preventing the trauma her family endured is worth any extra scrutiny.

"My gut feeling is if you want to be a donor, you’re doing a wonderful thing, but you also have to think about what could happen to the recipient," she said. "If I’m willing to be a donor, I’m willing to answer any of those questions that someone is going to ask of me."

The proposed guidelines shorten the time frame for many of the higher-risk behaviors from five years to one year. But they also classify as risky people who have used kidney dialysis during that time; people who have snorted cocaine or heroin nasally; those who've been in prison, jail or juvenile detention centers for more than three consecutive days in the past year; those who currently have or who have been treated for syphilis, gonorrhea or genital ulcers in the past year and people who have immigrated to the United States within the last year from a country with a high prevalence of hepatitis B.

Other aspects of the plan have drawn fire from transplant experts who object to tests that might be too expensive and too slow for all centers to administer.

But it's the new emphasis on two or more sexual partners that has ignited most ire, judging from public comments about the proposal being accepted through Dec. 21 at www.regulations.gov.

“I am opposed to the guidelines as written,” wrote Dr. John Radomski, chief of surgery at Our Lady of Lourdes Medical Center in Camden, N.J. “The list of high risk behavior seems way too broad.”

CDC officials downplayed the controversy, saying that the proposal is a draft and can be changed, particularly if there's strong evidence to support any alteration. They said the primary goal is to obtain as much information about transplanted organs as possible, whether that comes from personal histories or advanced screening tests.

Using a set of behaviors to gauge risk makes sense, Kuehnert said, and studies suggest that having more than one sexual partner raises the risk of infection.

“We can quibble about whether it should be two sexual partners or three or five or 10, but we’ll have to have a cut-off point,” he said.

Should donors who had sex with more than two people in a year be considered high risk? Tell us on Facebook.

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