Donor’s Selfless Act Carries a Painful Price

Giving the gift of life just about killed bone-marrow donor Lina Joy.

In a rare complication to an increasingly common procedure, Joy, 44, of Paramount got a staph infection in her marrow after the tissue was extracted from her pelvis at St. Joseph Hospital in Orange two years ago.

The ensuing treatment--including 65 days of hospitalization--brought her near death several times. She suffered liver, colon and kidney failure, and fell into a deep coma.

She is back working part time as manager of a Bellflower dental office, but her health has been unalterably damaged. She has an immune-system disorder, limp and speech defect. She has constant back pain and unexplained rashes.

To add heartbreak to injury, the recipient of her marrow died before Joy had even left the hospital.

Further compounding her woes, Lina Joy and her husband, Governor, 46, feared for months that more than $400,000 in unpaid medical bills would bankrupt them. Much of the financial fear was alleviated last month when the hospitals and doctors involved abruptly settled a medical malpractice suit the Joys had filed against them in 1997.

The case, however, has become a watershed for the tight-knit marrow-transplant community nationwide. Lina Joy’s near death was the most serious complication for a donor in the 12-year history of the National Marrow Donor Program, doctors who run the program say.

Though the risk of serious complications remains low, the Joy case became a wake-up call for doctors who recruit donors. The donor program--known as NMDP--was compelled to send a series of alerts, reminding recruiters to stringently outline the potential dangers. It also altered some of its recruiting material.

The case also prompted some consumer advocates to suggest that a good Samaritan law should hold donors free from liability for medical bills.

Critics and supporters of the donor program agree that the Joys’ ordeal emphasizes the inexorable tension between the need to gather the lifesaving marrow and the obligation to ensure that donors recognize the real risks in even such a simple surgery.

“Depending on what you tell them, there are some donors you will scare off and some donors you will unduly encourage,” said Dr. Peter Page, who heads the American Red Cross in Southern California, which runs donor programs for the NMDP. “If we were to minimize the risk, that wouldn’t be fair to donors who have complications. If we overemphasized the risks, that would hurt recipients.”

Many in Need Will Never Find a Match

There is no question the demand is great.

On any given day, 3,000 very sick people who have failed to find a donor among relatives are seeking a match among the 3.5 million people in the program’s Minneapolis registry. All of them have cancer or other terminal illnesses.

Many, perhaps 25%, will never find a donor despite remaining in the registry for months.

The NMDP is by far the largest of the marrow donor programs in this country. It assisted in more than 1,300 transplants in 1998. Since 1987, it has helped 7,900 people find unrelated donors. There are an additional 4,500 related-donor transplants a year.

Most people learn about giving marrow from the ubiquitous local drives that seek a match for a sick individual. Actually, the odds are infinitesimal that the sick person will match one of these volunteers, but it is an effective way to recruit donors for the bone-marrow bank.

Marrow transplants began to be widely used in the early 1980s to treat aplastic anemia and other marrow-related diseases. Today, about 75% of transplants are for victims of leukemia, or cancer of the marrow. But the demand for the procedure is growing as the technique has expanded to tackle hard tumor cancers, genetic disorders and some 40 other diseases.

For the recipient, transplantation is horrific and very risky. Essentially, patients are brought to the brink of death as their marrow is destroyed by massive radiation and chemotherapy. The donor’s marrow cells are then transfused into a vein and find their way to marrow sites, where hopefully they rebuild the immune system.

For the donor, the procedure is far simpler, commonly requiring at most one night in the hospital. Generally done under general anesthesia, the marrow--usually a quart--is extracted by a syringe and a large-bore needle called a trocar.

Patients are told to expect sharp discomfort in the small of their back. Most recover in several days, resuming full activity in two to three weeks. Complications are rare, and debilitating complications rarer still, said NMDP medical director Dr. Dennis Confer.

“The risk of a serious complication--an infection or an anesthetic reaction or lung problem such as pneumonia due to anesthesia, or an injury to bone from collection--runs around one to three per 1,000 collections in the last 20 years,” he said.

No NMDP donors have died. Of the four known fatalities among bone-marrow donors anywhere, three occurred overseas. The one in the United States involved an anesthetic reaction in a related donor, said Confer.

Marrow program literature intended for the public even today does not mention the fatalities or the Joy case, but everyone in the transplant community knows the story of Lina Joy.

“This was the most life-threatening case we have had,” said Confer. “It is the only situation where we were fearful that a donor might die.”

‘We Were So Excited About Doing This’

Inside her two-story house with its Dutch motif and tulips in the precise frontyard, Lina Joy works her hands together. She describes all that she has lost.

“I have pain every day and will be on medication for the rest of my life,” she says.

A swimmer who regularly did 50 laps in the backyard pool, she now manages a lap or two--and those only at night because of pigment damage from drugs used to treat her infection. “I am not allowed to go in the sun and I was a sun worshiper,” she says as her china-blue eyes brim with tears.

Her greatest fear on coming home was that she would remain an invalid. She couldn’t sit, dress or go to the bathroom alone. Her bed had been moved to the first floor. She set a goal of climbing one more stair each day.

A native of Holland who came to California at 4, Lina met Governor in high school. He retired in 1991 as a senior chief torpedoman on a nuclear attack submarine.

The Joys heard about marrow donation when their church held a drive for a local boy with leukemia. She wasn’t a match for him, but did fit a man in Australia.

“We were so excited about doing this,” she says, sitting in her pin-neat dining room, her meticulous make-up masking the blotches on her once-flawless complexion.

Within a day of going home from St. Joseph Hospital on Dec. 11, 1996, where the harvesting was done in conjunction with Children’s Hospital of Orange County, the Joys realized there was a problem.

Lina Joy had severe pain in her right hip and a high fever. She couldn’t move her right leg. She returned to St. Joseph with a staph infection that had taken root in her pelvis.

She would be hospitalized four more times--65 days over four months--and have surgery to repair bone destroyed by the infection. She was given massive doses of increasingly powerful antibiotics.

Those antibiotics took their own toll, provoking a massive reaction: Just before her final 40-day hospital stint that began March 2, she suffered chills as a vivid, dark purple rash covered her body. In the hospital, she was racked with coughing, her dermis separated from the underlying muscle in parts of her body, her tongue and the lining of her lungs peeled, and she fell into a deep coma.

Doctors puzzled over her body’s reactions and tried to devise a solution. They told Governor she was close to death three times.

The recovery is slow.

She sees doctors for chronic pain and the mysterious immune disorder. Along with the rashes, she has a permanent limp from the surgery and a slight speech impediment that appeared after the coma. She has little stamina and a lupus-like condition. She rolls up her sleeves on a blouse embossed with hearts and tulips to show some of the large blotches that cover her body.

“My family didn’t want me to look in the mirror for a long time,” she says.

Saving Patients While Protecting Donors

During early 1997, the marrow-donor program monitored Lina Joy’s condition. On March 27, the program sent a two-paragraph memo to its donor and transplant centers saying a donor had became “critically ill” from complications and reminding them “that we work in a complex field where there can be very serious risks.”

The memo prompted a rash of telephone calls from transplant physicians seeking more information, leading to a second memo a week later. The new three-page update gave more complete information about Lina Joy’s infection and detailed other instances of serious complications with anesthesia, infections and tissue injury.

“Prospective donors . . . must receive information that is realistic and balanced,” it concluded.

Transplant doctors walk a fine line between protecting donors and saving patients.

“There is a tension,” acknowledged Page of the Red Cross. “As physicians, we want to do no harm. But it would be a shame to overreact due to a single incident. There are many patients . . . who can only be saved through this.”

Critics contend that the program’s incremental recruitment process can lull donors and push them to participate. Typically, a person gives a little blood for genetic typing at a local drive, but doesn’t match that person. Only months later do they learn that they are a potential lifeline for a stranger who found them through the NMDP registry.

Subsequently, they are invited to an individual counseling session where they are given more information and asked to sign an “intent-to-donate” consent form.

If they do, the process becomes hard to stop: the recipient will have his marrow destroyed in preparation for the transplant, and the donor must follow through or the recipient will die.

Transplant physicians disagree about whether donors before the Joy case got adequate information in the NMDP-supplied literature and video. The program acknowledges that before 1997 this material did not mention serious complications at all.

To a large degree, what donors ultimately learn about risk, even today, depends on what they hear at the counseling session.

“I feel the warnings on the donor forms are insufficient,” said one doctor who runs a major center. The doctor, who spoke on condition of anonymity, said he does the critical counseling--rather than leaving it to a nonphysician coordinator--and tries to err on the side of scaring a matched donor.

“I tell people that there have been fatalities,” he said.

The two-page consent form signed by Lina Joy does discuss risk and includes this sentence: “Life-threatening reactions from bone marrow donation are extremely rare, however, the NMDP wants you to recognize that fatal complications during marrow donation could occur.”

Charles Wheldon, the Joys’ lawyer, calls it “inadequate, in particular in warning of a potential infection” from viruses or bacteria in a donor’s system. They “make it sound like it is a walk in the park,” he said, adding that the program puts pressure on matched donors to go ahead with the harvesting.

Confer rejects the notion that donors are ever pressured or led to believe there is little risk. “We try to make it very clear that it is not risk-free . . . and that death is not impossible with any kind of surgical procedure,” he said.

The NMDP made no change to the 16-minute video it sends matched donors. At one point, the video depicts a woman confronting the decision to donate. She signs as the image switches to a heart-tugging scene of a frizzy-headed preschool child in a plastic bubble awaiting a transplant.

It did update its literature, adding a very brief mention of “serious complications,” noting they “are rare.” There is no mention of the Joy case.

“We feel there is no reason to reveal details of what happened to her because it is not useful,” Confer said. “This kind of thing is so rare that this sequence of events is unlikely to happen again.”

Hospital Settled After Baby-Switching Case

For the Joys, one facet of the ordeal ended last month, when they settled the suit against the two hospitals and staff for an undisclosed sum and freedom from liability for unpaid bills.

The Joys contend that Lina got the infection from a nonsterile procedure during the surgery. The hospitals argue that it was caused by existing staph in her blood that was implanted in her marrow by the operation.

The case was settled just a week after St. Joseph was embarrassed by news that it had discharged an infant with the wrong parents. Several participants in the case agree that the desire to avoid publicity drove the settlement.

The hospitals maintain they were not seeking payment from the Joys, though the couple continued to receive doctors’ bills in 1999. The NMDP liability insurance--which covers donors--paid $250,000 before reaching its policy limit.

What doesn’t end, however, are the other impacts on the Joys.

“There is a lot more fear in my life,” she said. “Fear about what this has done to me and how it has shortened my life.”

The Joys say they hope that good will come from their ordeal.

They enthusiastically support suggestions that California adopt a good Samaritan law to limit donor liability for medical bills.

Lina Joy offers her own gift to future matched donors. She advises them to really focus on the decision when they sign the intent-to-donate form--rather than later.

“You don’t understand the risks until the day you go to the hospital,” she said. “By then, when you realize the gravity of the situation, the patient has already had 10 days of chemotherapy and if you change your mind that person will die. Who could turn their backs at that point?”

Lina Joy still finds “the blessings” in her experience and says she would encourage her adult children to be donors.

“I believe that life is a miracle and we don’t live for ourselves,” she said. “You cannot be a lone ranger. If you can help somebody by doing something as simple as giving a small piece of yourself, then you cannot deny a person their only chance at life.”