Gift of Life, Questions of Liability

Somewhere out there, in the sprawl of greater Los Angeles, the man who gave 8-year-old Brittany Johnson an incurable kidney disease may dwell.

At least, that’s what Brittany’s family suspects. They have no way of knowing, not yet. They don’t even know his name.

He is, to them, still a number: “Donor 276.”

As a 24-year-old law clerk in 1986, he walked into the California Cryobank in Century City to donate his sperm for pocket money. On the pages of a lengthy questionnaire, he reported few personal or family health problems--save for Page 9. There, an “X”--scrawled by him or an interviewer--indicates that his aunt had kidney disease, and an “X?” suggests a question about whether his mother did as well.

Eleven years later, this young man’s genetic confidences are at the crux of an unusual lawsuit pitting a Santa Barbara family against a sperm bank that portrays itself as an industry leader in donor screening.

The Johnson family, in its lawsuit, blames the bank for allowing someone with a family history of kidney disease to donate sperm--a blunder, they say, that has pared their daughter’s activities and may well cut short her life.

Vowing not to violate the privacy of the intimate exchange between donor and recipient, the bank has refused to discuss the case beyond denying wrongdoing.

Yet the lawsuit is about more than Brittany: It challenges the practices of one of the nation’s largest sperm banks, headed by physicians who for years have pushed for greater accountability in the infertility industry. It may serve, or so the Johnsons hope, as a reminder to infertile couples that, even as the industry seems to spin miracles, things can go terribly awry.

And it raises more general questions that make ethicists squirm: When are the risks of combining this egg with that sperm too great? To what lengths should tissue banks go to detect possible genetic defects? The natural rate of birth defects is 2% to 5%; should a sperm bank do better?

If precautions fail and a child is born with a serious hereditary defect, does the bank have a responsibility to warn others who used the same donor?

As more than a hundred thousand American women each year seek donor sperm and as more genetic flaws become detectable through tests, the answers become both more important--and elusive.

It doesn’t help, some critics say, that the infertility industry, with revenues of more than $2 billion annually, is largely unregulated. With the exception of infectious disease testing, safety measures are mostly voluntary in California, left to the discretion of professional societies and individual practitioners.

In the case of Donor 276, the issues are pressing and painful. According to court records, the man produced 320 vials of sperm that, theoretically, were available until he was “retired” in 1991.

If he carries the potent gene suspected in Brittany’s disorder, known as adult polycystic kidney disease (APKD), there is a 50% chance that any child conceived from those vials would develop some form of the disease.

Though APKD often remains hidden for years, surfacing only in middle age, doctors say it is a good idea for any at-risk patient to monitor blood pressure, avoid contact sports and make other lifestyle adjustments.

Brittany’s mother, Diane, wonders: Shouldn’t potential carriers be told so they can make those adjustments, or at least before they pass the gene on to their own children?

“Some people may think, ‘I never wanted to know this,’ ” she said. “I don’t want to bring a lot of sadness into [another] family. I don’t want to hurt or upset them, but as a mother . . . I would want to know.”

An Answer to Prayers

Ron and Diane Johnson describe themselves as reluctant messengers. They are private people, they say, and seeking donor sperm was their deepest secret. For seven years they did not tell anyone, even their parents, how Brittany was conceived. After her birth, they never even mentioned it to each other.

In fact, neither of them knew for certain, until recently, that Ron was not Brittany’s biological father. That is because his sperm was mixed with the donor’s during the fertilization process. But a paternity test done last fall as a result of the lawsuit concludes that Brittany was not conceived from Ron’s sperm.

The Johnsons say they recently have realized that the secrecy surrounding their daughter’s conception has served more to harm than to shield them. Diane, a pediatric nurse, believes it has protected a poorly regulated industry, allowing principle to be quietly sacrificed to profits.

“They don’t see that they are dealing with potential life,” she said. “It’s a business. That’s all.”

It is a business that, at first, seemed to answer their prayers. For years they had tried, and failed, to conceive a child. Diane couldn’t stand to look at a pregnant woman on the street. Ron felt he couldn’t console her or himself; his infertility was the problem.

Then, their hopes were awakened. On the eve of their third infertility procedure, the couple made a last-minute decision to use donor sperm mixed with Ron’s. Diane says she reviewed pages of donor descriptions from California Cryobank, settling on No. 276 after her first choice proved unavailable. Athletic, with dark hair and eyes, he seemed much like Ron.

Diane remembers a reassuring brochure, pledging genetic screening of the donor going back three generations. Beyond that, she said, “I didn’t even know what to ask.” So she bought two vials of sperm at $85 each.

Brittany was born in April 1989. She was a lively child with a knack for drawing unicorns, spotting shiny rocks and swishing Hula-Hoops from her waist to her neck.

The Johnsons were so taken with her that they sought to conceive another child, using the same donor, in late 1991. But Donor 276 was not available. According to the deposition of a Cryobank genetic counselor, he had been “retired” because of “new medical information.”

A document prepared by the counselor indicates that the bank discovered in 1991, after re-questioning the donor, that he had a far more extensive family disease history than he originally reported.

His maternal grandmother died of APKD and heart failure at 49; his 63-year-old maternal aunt, who also had the disease, had a kidney transplant; and his mother, 57, had APKD but was in “good health,” according to the document.

Prepared in response to questions from Brittany’s doctors in 1995, the document states that the donor himself was in good health in 1991, with no evidence of severe kidney dysfunction. And that is what Cryobank told Diane Johnson in late 1991--that despite the family history, an ultrasound on the donor was “clear,” according to Diane’s deposition.

Yet, in other court documents, Cryobank attorneys say the 1991 ultrasound--ordered by the bank after details of the donor’s history emerged--showed he had multiple cysts on both kidneys. While bank officials did not consider the ultrasound conclusive, the Johnsons and their attorney, Walter Koontz, contend that other medical records that the bank calls confidential would confirm whether the donor has the disease.

APKD is a progressive disorder passed from parent to child, though in rare instances it can be acquired through a mutation. Relatively common, it afflicts 1 in 1,000 people. Only one parent need have the defective gene to pass it on. Roughly half of those with the condition experience kidney failure before age 50.

In Brittany, the disease struck early. In 1995, she got an intense stomachache in gym class, then excreted blood in her urine. After detecting clusters of cysts on her kidneys, doctors diagnosed APKD. Given Brittany’s age, they predicted an “aggressive course.”

For now, it seems little more than an inconvenience.

One day this summer, Brittany burst into her family’s well-kept home, boasting of front flips and other swimming pool feats. Her 4-year-old brother, Brandon, conceived with the help of another Cryobank donor, whooped and squealed behind her.

Between displaying her crayon art portfolio and showing off her rock collection, Brittany talked matter-of-factly about the disease she already knows, in technical terms, as her “polycystic kidneys.”

It’s “not too bad,” she said, reassuring her visitor. “Sometimes it’s fun,” like when she gets to fly to Denver to see a special doctor. But she doesn’t like getting her blood drawn, and she “can’t run so much, or sometimes it hurts really bad.”

Her parents are trying to enjoy the here and now. But sometimes, they say, they can’t help but wonder what awaits her: Kidney failure? Dialysis? A transplant?

How can they explain it to her? How, with insurance covering only part of her care, can they pay for it?

The worst part, said Diane Johnson, is this: “I’m going to live to see her start to suffer.”

Bank’s Screening Process Described

Dr. Charles Sims is a soft-spoken, grandfatherly man with an impressive resume and a beeper that won’t leave him be.

Former chief pathologist at Century City Hospital, former director of a large national laboratory, an advisor to the state on tissue banking regulations, he is also a defendant in the Johnson case. In a 1986 interview, records show, he vetted Donor 276.

He won’t talk about the case, citing his commitment to maintaining the donor’s confidentiality.

But, he is passionate on this point: “We are here to help people. . . . What we’re doing in sperm banking is, we’re helping make families possible. It is literally the gift of life. Do we want to make it so litigious that can’t happen? I don’t think so. . . . Should we be accountable? Of course. But you can’t have things 100% safe. You can’t. You would [have to] ground every plane in the sky!”

Sims, 64, ticks off the bank’s key safety measures: Freezing and quarantining donor sperm; testing the donor regularly for HIV and other infectious diseases; taking a detailed donor history; testing for certain genetic defects; adhering voluntarily to the standards of professional societies. Of those who apply to be donors, he said, only 5% to 8% are accepted.

Even back in 1986, when sperm banking was in its infancy, California Cryobank was doing far more than others in the industry, he said.

But “nothing is perfect,” he said, stressing that the healthiest people are born with several hidden, but potentially lethal, genes.

There was no genetic test for APKD in 1986; even now, though a test is available, it is not routinely done. At California Cryobank and other banks, tests now are done for such genetic threats as cystic fibrosis, sickle cell anemia and Tay-Sachs disease, when appropriate. Still, much depends on what the donor says about his own heritage.

Donor 276, at first, gave partial or incorrect information, conveying to Sims that his aunt had an unspecified kidney disease, and that his mother might have it. The mother and an aunt or uncle also had high blood pressure. At the time, the donor apparently believed, erroneously, that his maternal grandmother had died of cancer.

Sims, as a donor interviewer, is not--and is not required by law to be--trained in genetic counseling. But the Johnsons’ attorney cites his lack of expertise as a problem. (The bank since has retained genetic experts.)

Outside medical experts said that, in general, high blood pressure and unspecified “kidney disease” in a donor’s close relatives would prompt them to explore the possibility of an inherited disorder.

Not doing that was like “running a flashing red light,” said Dr. Greg Rosen, the Long Beach fertility specialist who performed the Johnsons’ fertility procedure. He said he considers California Cryobank--now based in Westwood--an “excellent” facility overall, but, in this case, “Somebody got sloppy.”

Donor 276 supplied 320 vials to the bank over four years, according to court records (earning $35 per donation). One or two vials are generally used per attempt at conception, but experts said it is impossible to estimate how many children may have resulted.

Whether the donor’s progeny include one child or many, Sims said in his deposition that he does not believe any recipient, besides the Johnsons, has been told of Donor 276’s family history of kidney disease.

Doctors Say Family History Is Needed

Although much about Donor 276 remains a mystery, this, at least, is known: In 1986, he pledged a commitment to “personal responsibility.”

“I believe I must be the most honest, truthful, diligent, trustworthy and sincere person I can,” he wrote in slanted, tidy letters on his donor questionnaire. “One must be responsible to himself and to others to achieve quality of life.”

These words led Diane Johnson to speculate this summer that the donor would want to help Brittany. But a Cryobank official, in a recent deposition, said the donor, when informed in general terms of the lawsuit, insisted on keeping his identity secret.

The couple say they bear “276” no ill will and do not intend to sue him. But in addition to seeking monetary damages in their lawsuit, they also seek to learn the donor’s identity. They believe they have been misled by those who screened him--and that they have a right to talk to him directly.

Two doctors declared in court documents that the donor’s medical history is needed to predict the course of Brittany’s disease-- whether, for example, she is likely to suffer the potentially lethal complication of brain aneurysms.

Initially, the courts backed the Johnsons. In April, a Los Angeles County Superior Court judge ordered the sperm bank to answer their attorney’s questions about the donor.

“Both public policy interests and the compelling health needs of the minor child . . . outweigh the privacy interests of Donor 276,” the judge said.

But that decision is now under reconsideration after an appeal by California Cryobank. The bank’s contract with the donor assures him of confidentiality unless a court orders otherwise.

Ethicists said there may be some compromise possible--perhaps releasing necessary information short of identifying the donor. Others said further exposure of this donor may scare others away, leaving infertile couples with fewer options.

The real danger, said Boston University ethicist George Annas, is the shameful hush around such donations.

“We’ve got to stop treating this like some little dirty secret. . . . There’s nothing wrong with it. . . . The most important thing is not keeping the donors’ identity a secret--it’s taking care of these kids. That’s got to be the first priority.”

Several ethicists flinched at the notion that other recipients had not been notified of the donor’s history, saying families should be spared the possible anguish, and expense, of piecing together a puzzle of symptoms.

But others countered that since the disease is incurable for now--and may not occur at all--the news would do little more than cast a pall over young lives.

There are other gnawing concerns. If virtually everyone is carrying some potentially deadly genes, how far should tissue banks go to detect them in donors?

When children are conceived naturally, the partners often know far less about each other’s genetics than a bank learns about a donor. Yet, because sperm banks screen the donor for the recipient, and the dealings are financial rather than romantic, the public often expects the bank to meet a higher standard than “nature.”

Industry members say donor sperm has never been safer. But laws governing sperm banks--set by each state--are “quite sparse,” said Ronald M. Harkey, a supervisor of tissue bank licensing for the state who is overseeing development of tighter regulations.

Since 1992--late even in Harkey’s estimation--sperm banks in California have been required to screen for HIV and other infectious diseases and to develop procedure manuals. Still, there are no requirements for genetic testing. Most screening measures are voluntary, although the American Assn. of Tissue Banks accredits organizations--including California Cryobank--that meet its detailed standards.

Though some physicians chafe under government regulation, ethicists and others increasingly are calling for greater oversight of the infertility industry. Recent scandals--including allegations of human egg theft by doctors at UC Irvine Medical Center--have given the issue a political shove.

A new treatment for male infertility may reduce demand for donor sperm. But the procedure is costly, often putting it out of reach. And improved prospects for freezing eggs will enhance the importance of female donor screening.

No one argues that genetic testing is a panacea. It too is costly, and no amount of money can buy a guarantee.

“Even when we have the whole genome mapped and we have tests for every [known] genetic disorder, there is no way we can say to a couple . . . that they will have a healthy child,” said Mary Mahowald, a University of Chicago ethicist. “Unfortunately, the hoopla over genetics has made people think we can have that certainty.”

The Johnsons say they weren’t looking for certainties, but their daughter’s illness was no low-odds accident. Brittany, they say, was given half a chance from the start.

“I just wish they would really follow the screening process,” Diane Johnson said. “I’m not telling people not to do this [procedure]. I have two beautiful children from it. But [276] should never have been a donor.”

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Precautionary Tips

Those seeking donor sperm from a bank should:

* Get as much information about the donor’s health, personality and appearance as possible.

* Ask for results of any specific disease or genetic testing of donors. Screening for hepatitis B and C, HTLV I, syphilis and HIV are required by law. Ask about the donor pool and where donor advertisements are placed. (Banks commonly seek college students in good health.)

* Ask about availability of genetic counseling to match donor and recipient.

* Ask for a tour of the facility to determine how specimens are collected, processed, stored, tracked and distributed.

* Ask how long specimens are kept and when they are discarded. Don’t buy specimens that have been frozen and thawed more than once.

* Check the bank’s state license to make sure it is in good standing. Also, check whether it is accredited by the American Assn. of Tissue Banks, and whether its medical professionals are members of the American Society for Reproductive Medicine.

* Check qualifications of staff members and obtain resumes of key technical staff.

* Ask about the bank’s policies on donor and patient confidentiality. Review blank consent forms for recipients and donors.

Source: California Department of Health Services