PROSPECT — Patricia Zappone knows the pain of hopes dashed since she learned she has terminal liver disease.
She hoped her husband could donate a portion of his liver and save her life. He proved not to be a match.
Zappone, 65, of Prospect, toyed with the idea of moving to Georgia, where the waiting period for a liver is significantly shorter than in the Northeast. She couldn’t afford to move.
Her physician told her the odds of her getting a transplant off the list are “diddly-squat.”
She is not alone. In 2013, 1,523 people died — an average of eight a day — while waiting for a liver transplant, according to the United Network for Organ Sharing, which manages the waiting list. Another 1,552 people were removed from the list, deemed too ill to receive a transplant.
A movement to revamp the organ donor allocation process for livers is gaining momentum. Even if reforms were to take place, it likely would be too late for Zappone.
“It seems I am not sick enough yet and, if I do get sicker, then there would most likely not be a liver available,” said Zappone, sitting on a couch in the tidy living room of her modest ranch home.
Zappone, who has short, silver hair and seafoam-green eyes, looks well, though she fatigues easily.
“Very sick people die waiting and Steve Jobs flies to Texas and gets right in,” said Zappone, of the Apple founder who received a liver transplant in 2009, but died in 2011.
Jobs was listed at multiple transplant centers and had a private jet to reach them in a timely fashion.
Listing at multiple centers is not so much the problem as the algorithm for donation, said Billy Wynne, an adviser with the Coalition for Organ Distribution Equity, a Washington, D.C.-based nonprofit dedicated to improving patient access to organs for transplant, particularly livers.
“The current system is disproportionately based on geography, rather than patient need,” Wynne said. “Where you live can dictate how long you wait, and ultimately, whether you live or die.”
The country is divided into 11 transplant regions, which were developed based on existing relationships between transplant centers. They were not designed for “optimal organ distribution,” concluded a 2014 report by the organ network committee studying the problem.
The committee’s answer: redistricting. Reducing the number of transplant districts to eight could save 330 lives a year, according to the report. Reducing it to four would save more than 550. Increasing district size would level the playing field because it would combine areas with low donor supply with those that have a plentiful supply, said Dr. David Mulligan, a transplant surgeon and director of the Yale-New Haven Transplantation Center.
Getting a liver transplant in the Northeast or on the West Coast is much more difficult than it is in other parts of the country. Residents of the South, Southeast and central United States are more likely to die at younger ages, Mulligan said.
“There are more strokes at a younger age (in those areas) and more trauma related injuries from automobile and ATV accidents,” Mulligan said. “Here, people tend to live longer and when they do pass away, it’s from cardiovascular disease and cancer and other things at an age range like the 80s when the use of organs is very slim.”
While the use of seat belts and motorcycle helmets is likely a factor, Mulligan said dietary habits and lifestyle also matter. The stroke belt, he pointed out, is an 11-state region across the south where the risk of stroke is 34 percent higher than in other areas of the country. The geographic disparities of the current system mostly affect livers, Mulligan said.
Zappone suffers from nonalcoholic steatohepatitis, known in medical circles as NASH. It resembles liver disease caused by alcoholism but occurs in people who drink little or no alcohol. She is eligible to receive a transplant at either Yale-New Haven Hospital or Hartford Hospital.
Liver transplants are ruled by something called a MELD (Model for End-Stage Liver Disease) score, which determines where patients rank on the waiting list. The sicker the patient, the higher the score, which ranges from six to 40. A person with a MELD score of 40 has a 10 percent chance of living for 90 more days. Zappone has a MELD score of about 25.
In the Northeast, most patients need to have a MELD score of 33 to 35 because the organs are so few and they need to go to the sickest patients first, Mulligan said.
“That is one of the highest in the country and the death rate on the waiting list here is one of the highest in the U.S.,” Mulligan said. “In Kansas, Alabama, Georgia, Texas, Louisiana, Florida, patients get organs with MELD scores in the mid-20s. Certainly, these people need transplants, but they could wait a little bit longer and let those organs go to the patients who are literally days from death.”
Dr. Simona Jakab, a Yale gastroenterologist and transplant hepatologist who has treated Zappone, said she welcomes changes to the allocation system.
“The mortality on the waitlist can be as high as 30 percent,” Jakab said. “It’s heartbreaking.”
The proposal has met resistance from congressional delegations in Georgia and Iowa, Wynne said.
“There are winners and losers … no one would be truly harmed, but people in those areas would have their wait lists grow,” he said. “We have to look at this as a country and be altruistic, rather than provincial.”
At 41, Zappone was denied coverage for a life insurance policy when a required round of blood work showed markers for liver disease.
Her primary-care physician was unimpressed with the results and told her they would keep an eye on it. Eating healthy and exercising regularly may help prevent liver damage or reverse its early stages, but once the liver swells and becomes heavily damaged, transplantation is the only option.
Zappone felt fine until she began to notice red spots on her face, neck, shoulders and back in 2005. In 2007, she was diagnosed with NASH, a fatty liver disease. It can range from just fat in the liver that causes no symptoms to fat with inflammation. That inflammation can lead to scar tissue, and when there is too much of that, it is cirrhosis, Jakab said.
“Without transplant, people with this will die,” Jakab said. “The median survival time is about two years from the time of diagnosis.”
Zappone, who spent her career in banking and income tax jobs, stopped working about 10 years ago. It was hard to keep up with all the medical tests. Plus, she has struggled with encephalopathy, a loss of brain function that happens when the liver is unable to remove toxins from the bloodstream. As a result, Zappone has fallen into several comas.
The prognosis is stark, but she is determined to see certain things through, including her only child’s upcoming marriage in the fall.
The liver, the largest organ in the human body, can regenerate completely. It and the skin are the only two organ systems in the body with that ability.
That makes a living-donor transplantation a strong option for someone like Zappone. If a prospective donor was a correct match, a transplant could take place immediately, rather than waiting until she is more ill and therefore higher on the waiting list.
A patient in stronger health would also have a much better recovery, Mulligan said.
But asking friends and family members to undergo a major surgery for her sake is difficult for Zappone, a private woman.
“It is so hard for anyone to give up the time to endure surgery. I completely understand that, but getting the message out there could help others, even if it might not help me,” she said.
At Yale, patients are encouraged to use a donor champion, “someone from the family to advocate and ask friends and family to consider undergoing surgery,” Jakab said.
Only 300 living liver donations are done a year in the United States, Mulligan said, and Yale averages between 10 and 17 a year.
While a liver transplant is a complicated surgery, the risk to a donor is the same as going for a ride on a motorcycle with a helmet, he said.
“The risk of dying as a living liver donor is .2 percent or 2 out of 1,000 so it’s very, very low.”
It does, however, require an incision underneath the ribs, whereas a kidney transplant can be performed via laparoscopy.
Mulligan said he is working to grow a paired donation program for living liver donors.
“If someone calls and says they want to be a donor for their sister or brother but find out they are the wrong blood type, before, we stopped the process right there,” he said. “But now we can say, ‘Great, we have another pair who has the opposite problem,’ and we can do a two-way swap and it starts this chain effect. We did an eight-way swap of kidneys last year, all in the same day.”
The benefits of a living donation are manifold, he said.
“It can be a lifesaving gift and turn someone’s life around in a couple of days for the recipient. And they can have a better outcome not only immediately after the transplant, but we even have data now that shows at 10 years, we have statistically better outcomes than we do with a deceased donor,” Mulligan said.
For more information, visit Donate Life Connecticut at ctorganandtissuedonation.org or Donate Life America at donatelife.net.
For information on the living donor program at Yale-New Haven Hospital’s Transplantation Center, call (866) 925-3897. For information on Hartford Hospital’s program, call (860) 972-4219.