THE VIRGINIAN-PILOT
Copyright (c) 1997, Landmark Communications, Inc.
DATE: Thursday, January 2, 1997 TAG: 9701020051
SECTION: FRONT PAGE: A1 EDITION: FINAL
SOURCE: BY DEBRA GORDON, STAFF WRITER
DATELINE: PORTSMOUTH LENGTH: 161 lines
Kim and David Gosey's 5-month-old daughter Hannah is dying.
But the Portsmouth couple don't want your pity. They don't want your money. They want your bone marrow.
Hannah Gosey, a red-haired infant with pale blue eyes, suffers from a rare genetic defect named Hurler's Syndrome. Her body doesn't make the enzyme necessary to break down sugar molecules with the tongue-twisting name of mucopolysaccharides. Without that enzyme, the jellylike substance accumulates in Hannah's cells, causing progressive damage to her brain and other organs.
Today, Hannah looks and acts like a normal baby, scooting across the Gosey's small living room, gnawing on the giant pink elephant she got for Christmas.
But by the time she starts elementary school, she will be severely retarded, blind and probably deaf. Chances are she will be in a wheelchair, her bones twisted and bent. It is doubtful she will live long enough to start fourth grade.
Unless she gets a bone-marrow transplant.
That's where you come in.
Hannah has a one-in-a-million chance of finding a bone marrow match through the National Marrow Donor Program. Bone marrow - the spongy substance in the middle of bones that manufactures red blood cells - must be a nearly perfect match at the DNA level before transplant.
Matching is a game of numbers. The more people who become part of the registry, the greater Hannah's chances - and the chances of the other 24 Hampton Roads residents waiting for a bone-marrow transplant - of finding a match.
So the Goseys have organized a bone-marrow drive for Sunday. They hope that, by adding hundreds of names to the national registry, they can improve the odds of Hannah finding a match.
It's not a simple thing they're asking you to do. Donating bone marrow is not like donating blood. First, there is a $19 fee just to get your blood typed. The normal fee is $45, but, during special drives like Sunday's, the Red Cross waives part of the fee.
Second, you are not just having your blood drawn to see if you are a match for Hannah. You're agreeing to have your tissue type and name added to the national registry. Two thousand people search every day for a match, and 30 to 40 a day die waiting.
So once you are on the registry, you could get a call from, say, California, asking you for your marrow. It would mean undergoing surgery, in which doctors push a needle into your hip bone to extract several cups of the precious tissue. For a couple of days after the procedure, you would be sore, as if you'd fallen off a horse.
Before you make up your mind about showing up Sunday, there's more you should know about Hannah Gosey.
Hannah sounds like she has a cold. Her nose is stuffed, she is sniffling and snorting, and the first thing you want to do when you meet her is run for the decongestant.
Don't bother.
Chronic congestion is one of the symptoms of Hurler's Syndrome.
The syndrome is so rare that most pediatricians know about it only through books. Certainly Hannah's doctor, Dr. Sandra Baucom, had never seen a case. But she knew something was wrong with this baby. By the time she was 3 months old, Hannah was a regular in Baucom's Chesapeake office, constantly in for colds, coughs and vomiting.
She also wasn't growing. Baucom and her partner suspected reflux, a condition in which Hannah threw up nearly every time she ate. They sent her to Children's Hospital of The King's Daughters for an X-ray. That is when an observant pediatric radiologist noticed something funny about Hannah's vertebrae.
Bony deformities are another symptom of Hurler's Syndrome.
A referral to pediatric neurologist Matthew L. Frank resulted in the diagnosis. On Dec. 3, Frank told the Goseys their daughter had an incurable disease with a life expectancy of age 10.
Then he got on the Internet.
Medicine today changes so fast that it is almost impossible for doctors to keep up with every advance for every disease. Sometimes, a little research is required. It didn't take Frank long to find that in the past couple of years, a cure for Hurler's had been found - a bone-marrow transplant.
It was a cure that itself could be deadly. A bone-marrow transplant involves killing off Hannah's own marrow with near-lethal doses of chemotherapy, leaving her vulnerable to infections. After the transplant, she runs the risk of contracting graft-vs.-host-disease, a potentially fatal process in which the new marrow attacks her body.
But given the certain death Hannah faced without the transplant, it was a risk the Gurleys were willing to take.
In a way, Hannah was lucky. Most children aren't diagnosed until they are 3 or 4 years old, when they begin showing signs of mental retardation. By then, the damage to their brain, limbs and other organs is usually severe.
``It's a miracle they diagnosed her so early,'' Baucom said.
The Goseys try not to think about the incremental damage that every passing day inflicts on their daughter. They have chosen to cope with this disease through action instead of paralyzing sorrow.
Kim, 27, a medical insurance specialist who works for Baucom, and David, 29, a boilermaker at the Norfolk Naval Shipyard, organized the bone-marrow drive just two days after learning about the transplant, even before they located a hospital to do the procedure.
Since then, they've been accepted into the transplant program at Duke University Medical Center, learned their insurance will cover the estimated $100,000 cost, even found a campground where Kim can stay while Hannah spends what may be months in the hospital.
They have had their blood drawn to see if, by some miracle, they are a match for Hannah. It is doubtful. Together, they make up Hannah's whole; separate, they provide only half a match.
Hannah is their first child, so there are no siblings to test. Siblings often provide good matches for bone marrow transplants, but the odds are 1 in 4 that the Gosey's next child would have Hurler's Syndrome.
And while the Goseys have a large extended family who will be tested Sunday, chances of a match are slim.
They are counting on the marrow of strangers.
``We're not out just for us,'' said Kim, as Hannah slept on her chest, sucking noisily on her pacifier. ``We hope this drive will also help someone else.'' ILLUSTRATION: Color photo by TAMARA VONINSKI/The Virginian-Pilot
Hannah Gosey, who suffers from a rare genetic defect called Hurler's
Syndrome, is held by her parents, Kim and David Gosey.
Photo by TAMARA VONINSKI/
Kim and David Gosey share a quiet moment with their 5-month-old
daughter, Hannah, who has a rare, fatal genetic defect named
Hurler's Syndrome.
Graphic
IF YOU WANT TO HELP
The bone-marrow drive for Hannah Gosey will be held Sunday from 1
to 7 p.m. at Dr. Sandra Baucom's office, 4012 Raintree Road,
Chesapeake, near Chesapeake Square Mall. It costs $19 for Caucasians
to have their blood tested and names placed on the registry.
Minorities, including African-Americans and Filipinos, are free.
Potential donors must be between 18 and 60, and must be in good
general health.
Donors are asked to sign a consent form and give a brief medical
history before giving a vial of blood - about four tablespoons. The
information about the donor's marrow type is then logged into the
computerized National Marrow Donor Program.
The actual marrow removal is not done until a match has been
confirmed through further testing and the donor agrees to the next
step of the procedure. Then a Red Cross representative meets with
the donor and assists with plans for a hospital stay, during which a
small amount of bone marrow is removed from the hip. There is no
cost to the donor for the hospital procedures.
For details, call the Red Cross, 631-2050.
Graphic
PAY TO VOLUNTEER
So you decided to be a good Samaritan and get your blood
tissue-typed to add your name to the National Marrow Donor Program.
That will be $45, please.
Wait a minute. Why should you have to pay for the privilege of
volunteering?
Because the cost of tissue typing - in which certain substances
in the blood called antigens are identified - is expensive, said
Andrea Carter, public relations specialist for the Minneapolis-based
National Marrow Donor Program. The actual test costs between $300
and $500.
``It's a very detailed process compared to just finding out the
type of blood,'' Carter said.
The Red Cross has funds earmarked for community drives like the
one Kim and David Gosey have organized for Sunday. Minorities,
because they are in such demand on the registry, have their fees
waived all the time, thanks to a special grant from the Navy.
KEYWORDS: BONE MARROW TRANSPLANT INFANT HURLER'S SYNDROME