Sister's Sacrifice : Before transplant, weeks of unease[0]

    Spared a hereditary kidney disease that has devastated her family, a
woman embarks on a risky journey to liberate her sister from it

    By Alice Hohl[0]

    Staff writer


    Part one in a Daily Southtown series


    Deciding to donate a
kidney is difficult enough.

    If you have lingering worries that you or your children could be
struck by a hereditary kidney disease, the decision is harder still.

    For Suzanne Ruff, nothing about donating a kidney to her sister, a
teacher's aide at Oak Forest High School, is easy.

    Jo Ann Villanueva, who needs her sister's kidney to avoid waiting
several years on dialysis, gets teary-eyed when she talks about her
sister's sacrifice.

    "If there was only another way," Jo Ann said, looking over at her
sister after a hospital consultation last week.

    Jo Ann said she is uncomfortable with being in the limelight but
hopes by sharing her story, more people will become organ donors when they
die so fewer living donations will be required.

    If Suzanne were unable to donate, Jo Ann would face a four-year wait
for a kidney from a cadaver. She has three four-hour dialysis sessions per
week and uses a catheter because her kidneys don't produce urine.

    She is limited to 32 ounces of water or liquid per day and can't eat
many foods.

    Jo Ann dreams of the day she can drink until she isn't thirsty
anymore, and said when she comes out of the surgery, she wants chocolate
and gallons of ice-cold water.

    A family stricken

    Fourteen members of the women's extended family have polycystic
kidney disease, as far as relatives know.

    In the Gill family, the mother, Joan, had the disease, as well as her
brothers. Joan Gill passed it on to two of her three daughters.

    The Gills raised their daughters in Evergreen Park, the three girls
moving through Most Holy Redeemer grade school and Evergreen Park High
School.

    Of the three, the youngest was diagnosed first.

    When Joan Gill's brothers began to sicken and die from kidney
complications, Joan and her daughters, then teenagers, went for testing.

    Although the tests showed Joan and Janice were both likely to develop
it, Joan and her husband told the children everyone was healthy.

    "It would have been rough for a young girl to know she had a disease
like that," John Gill said. "We told them that everything was fine."

    Not until Joan collapsed on a golf course and was put on a regimen of
dialysis did Joan and John sit down with Janice, then 25, to tell her the
bad news: Janice also probably had PKD.

    Janice never developed the common symptoms of PKD: high blood
pressure, blood in the urine, frequent urinary tract infections and pain
near the kidneys.

    She was enrolled in a research study to determine the affect of
low-protein diets on PKD patients, and as a result, her kidney function
was tested every six months.

    "After about three years, the nurses told her that her kidney
function was dropping so fast they felt she should go home and get on the
transplant list," John said.

    "We went down to Northwestern ... because Joan had her transplant
there," he said.

    "The day before she would have started dialysis she got her kidney.
It was a miracle."

    Janice received a donor kidney Jan. 10, 1995, from a 12-year-old girl
who died in Oklahoma.

    Both of the older girls thought they had escaped the disease,
according to the best diagnostic testing in 1972.

    What is polycystic kidney disease?

    "Polycystic" means "many cysts." Diseased kidneys develop cysts,
enlarging the kidneys and eventually taking over, keeping the kidneys from
doing their work of filtering toxins from the blood and excreting them in
the urine.

    More than half of people diagnosed with PKD will have kidney failure
as a result. Once the kidneys fail, dialysis is the only treatment, and a
transplant is the only way out.

    In some PKD victims, the heart and blood vessels have been strained
by the disease, leaving victims prone to a heart attack, stroke or
aneurysm. Those who receive transplants and rely on immune suppressants to
keep kidney rejection at bay sometimes fall victim to other illness their
compromised immune systems could not ward off.

    For some people with PKD, slower growing cysts may allow them to live
a full life without kidney failure. Others are struck by the symptoms
later in life.

    Sentenced to dialysis

    For Jo Ann, who thought she did not have PKD, that day came Dec. 13,
eight months after her mother, Joan, died of a heart attack.

    Last December, Jo Ann, Suzanne and Janice joined their cousins for
their annual downtown Chicago outing. The sisters stayed in a hotel
downtown, shopping on Michigan Avenue and treating themselves to high tea
at the Drake Hotel.

    That Saturday, on the way back to her hotel, Jo Ann became ill and
began vomiting. She became so weak, her sister had to help her into her
pajamas.

    Jo Ann went home and saw a doctor Monday. By Wednesday, she was
sitting in a dialysis center for the first time, hooked up to a machine
that filtered her blood.

    And so Jo Ann joined the ranks of Gill relatives sentenced to
dialysis and a long wait for a transplant.

    Suzanne said the origin of disease in their family is a mystery,
since the disease was difficult to diagnose before ultrasound and
high-quality scans were invented. Nine of 14 relatives with PKD have died.

    "We don't know where it  started," Suzanne said.

    "We don't know where it's going to end, either," Jo Ann said. "But
we'd like to end it soon."

    The sisters were told the only sure way to prevent PKD from surviving
in their family was to avoid having children.

    By the time Jo Ann found out she had PKD, she already had two
daughters.

    Decisions

    Suzanne also has two daughters. All three were tested again for PKD
before she agreed to donate a kidney to her sister. Using more
sophisticated tests, doctors told Suzanne she was unlikely to develop the
disease, and her daughters probably do not have it, either.

    Still, there's a 1 percent to 5 percent chance the doctors and tests
are wrong.

    Suzanne's husband, Bill Ruff, said such statistics make him more
certain donation is the right choice for his family.

    "I'm a numbers guy, and statistically she's got a better chance of
getting killed in a car accident," Bill said of Suzanne's chances of
developing PKD.

    Still, Suzanne is not blindly optimistic about the transplant, which
will take place Oct. 19 at Northwestern Memorial Hospital in Chicago.

    She knows there is very little follow-up with donor patients, because
they were healthy to begin with and are not under the care of a kidney
specialist.

    She knows the national organization that keeps statistics about organ
donations does not track what happens to living donors.

    She knows she is not supposed to be fully recovered until three
months after the surgery, and yet there will be no follow-up appointments
after the first week.

    She has read voraciously about the risks and side effects.

    "I want to help her, but there's a whole aspect of donating that
hasn't been researched," she said.

    She wakes up sometimes in the middle of the night, terrified
something will go wrong or her daughters will end up needing a kidney,
too.

    But Suzanne said ever since the morning after Jo Ann got sick, she
has known deep down she would donate a kidney.

    "I knew there was a reason I didn't have (PKD)," Suzanne said. "I
knew there was a reason."

    Suzanne said being the only sister to escape the disease makes her
feel it is part of her destiny to help Jo Ann.

    "We're scheduled," she said. "I don't think there's anything stopping
us."

    Suzanne said she knows an end to dialysis and PKD symptoms will be
dramatic for Jo Ann.

    "It's going to completely change her life," Suzanne said.

    Jo Ann looked over at her sister.

    "And I hope it doesn't change her life at all," Jo Ann said.

   Part 2