North Hills News Record
Pittsburgh, Pennsylvania

Tuesday, September 29, 1998

"Mother's ordeal sparks labor of love"
Duo starts group to help people with hemochromatosis

By Gordon Ovenshine, Staff writer

Josephine Thomas's nagging symptoms would not go away. She felt fatigued.
Her hips ached. She couldn't get warm.

But seven doctors brushed her aside, saying exhaustion and sore joints went
with the territory for a 63 year old woman.

"Take some aspirin or Motrin," Thomas recalls being told.

Finally, a radiologist made the diagnosis. Thomas had hemochromatosis-a
serious condition in which the body absorbs too much iron. Affecting as
many as 32 million Americans, the genetically inherited disease can lead to
potentially fatal complications.

Thomas, along with her daughter, Sandra, started a crusade to educate
people about the disease and encourage annual screenings. In April, Sandra
Thomas, started the American Hemochromatosis Society. Her mother started
"Seniors HHelping Seniors".

"We're doing this as a labor of love," Sandra Thomas said. "We don't want
others to go through what my mother has been through."

At normal levels, iron is an essential nutrient for blood and muscle cells.
At toxic levels, iron damages tissues throughout the body and can spark a
number of ailments, including cirrhosis.

The problem with hemochromatosis is that other symptoms often mask the
disease. Chronic fatigue syndrome and diabetes are common manifestations
of hemochromatosis. In advanced states, the heart, liver, pancreas, and
reproductive system can be damaged, doctors said.

Although it is the most common genetic disease in the country, it goes
largely unnoticed by most Americans, said Steve Barfield of the Iron
Overload Diseases Association in West Palm Beach, Florida.

"It has just been neglected," Barfield said. "It's not covered by any one
medical specialty and it just fell through the cracks."

Treatment is simple but seemingly primitive-bloodletting.

In the initial phase, one unit of blood is removed once or twice per week.
The process can take as long as three years.

"It was a strange thing," said Josephine Thomas, now 77. "It would make me
feel grand. It's almost like your system knows you need to get rid of some

Researchers discovered the genetic basis for hemochromatosis two years ago.
Doctors said the gene is most common among people of British and Celtic
descent-the Irish, Scottish and English.

One's diet-too much red meat or vitamin C-does not cause hemochromatosis.

Dr. Geoffrey Block, a liver specialist with the University of Pittsburgh
Medical Center, said many doctors won't order a blood test as a precaution
because insurance doesn't cover it.

"With managed care, if anything, the routine measurement of iron status is
decreasing because of cost containment issues," Block said.

But doctors deserve some of the blame for iron overload and its subsequent

"It's not clear to me, why this has been ignored," Block said. "If you're
not thinking of it, you tend not to look for it."

North Hills News Record
Pittsburgh, Pennsylvania
November 17, 1998

"Health Center opens for blood disease patients"
by Laurie Cioffi

Getting treatment for her hemochromatosis often meant Josephine Thomas'
insurance didn't cover the cost.

But now the 77 year old Ohio Township woman will have a less expensive

The area's first center devoted solely to the diagnosis and treatment of
hemochromatosis has opened in Oakland. The Hemochromatosis Center is
affiliated with the UPMC Health System.

The clinic's director, Dr. Geoff Block, said many patients must go to
emergency rooms or outpatient clinics for treatment, but because their need
is not life-threatening, they often wait while others with more serious
needs take precedence.

"Some spend all day waiting for treatment that takes 15 minutes," he said.
"It's so much of a hassle that some people just don't do it, but it really
makes all the difference in the world."

Hemochromatosis is a serious condition in which the body absorbs too much
iron. The often genetically inherited disease can lead to potentially
fatal complications.

At normal levels, iron is an essential nutrient for blood and muscle cells.
At toxic levels, iron damages tissues throughout the body.

Thomas found that the most convenient source for the only
treatment-bloodletting-was a blood bank, but because she was giving blood
for a medical condition, she was charged $32 per visit.

At one point, she needed to have blood taken at least once a week.

Now, the new clinic will be a less expensive alternative and her Medicare
will pick up the bill.

Block's clinic also will diagnose the disease, offer genetic counseling and
nutrition education. He estimates about 10,000 people in this region have
the disease, but not all are aware of it. Blood and genetic tests can
detect the problem.

Left untreated, the problem can mask itself as many other diseases.
Chronic fatigue syndrome, and diabetes are common manifestations of
hemochromatosis. In advanced states, the heart, liver, pancreas, and
reproductive system can be damaged, doctors said.

Although it is the most common genetic disease in the country, it is now
more widely understood, said Sandra Thomas, Josephine's daughter and
president of the American hemochromatosis Society. Recently, the two
started groups that support senior citizens and children with the disease.

"We are thrilled (about the opening of the center). We are hoping this
leads to many people getting diagnosed," Sandra Thomas said.

North Hills News Record
Pittsburgh, Pennsylvania
Thursday, December 03, 1998

December 3, 1998
"An untapped resource"
Laure Cioffi
Josephine Thomas of Ohio Township has had 106 pints of blood drawn from her
in the past 15 years - but none of it went to people in need of transfusions.
Thomas, 77, has a genetic condition called hemochromatosis or "iron
overload disease" - a serious disorder in which the body absorbs too much
iron. The only treatment is phlebotomy or drawing blood from the patient.
To find out more
For details about hemochromatosis, call the Hemochromatosis Society at
(412) 635-8171; write to the American Hemochromatosis Society, 777 E.
Atlantic Ave., Z-363 Delray Beach, Fla. 33488-5352; or visit their Web site
About hemochromatosis
What: Hemochromatosis is an hereditary disease in which the body absorbs
too much iron, turning the necessary nutrient into a toxin.
Who: 32 million Americans are at risk; one in nine people is a carrier,
making it the most common genetic disease. Symptoms can develop in children
or adults between the ages of 20 and 60.
Early symptoms: High concentrations of liver enzymes, impotence,
irritability, depression, hair loss, joint pain and fatigue.
Effects: Gray or bronze skin pigmentation, cirrhosis, liver cancer,
diabetes, heart disease, death.
Treatment: Bleeding. One unit of blood is removed once or twice per week.
The process can take from one to three years. Thereafter, a person needs to
give blood about four times per year.
Prognosis: Normal lifespan if treated.
Source: Centers for Disease Control
Blood banks don't accept blood from people with hemochromatosis, even
though it is perfectly safe.
The blood bank has a policy against using the nearly 6,000 units of blood
collected each year from people in the Pittsburgh area with hemochromatosis.
Blood bank officials say using the blood could go a long way toward
alleviating the shortage in the blood supply.
But blood bank officials fear that because people who have hemochromatosis
are required to pay to have blood drawn, they might not be as forthcoming
about revealing risky behavior that could taint their blood, such as
intravenous drug use and unprotected sex with people who are HIV positive
or hepatitis.
"We'd love to get 6,000 more donors," said Dr. Joseph Kiss, medical
director of the Central Blood Bank of Pittsburgh. "If we could just get
around this issue, it would be an enormous windfall for the blood supply."
Nationally, about as much blood is drawn in a year from people who have
hemochromatosis as other donors. In 1996, the last year for which figures
are available, 14 million units of blood were donated.
Blood bank officials say there is less fear about receiving tainted blood
from people who volunteer to donate because they are doing it out of a
sense of responsibility.
"Our blood supply in the United States is built on volunteerism. It's
really a cornerstone of safety. Volunteer blood donors are motivated by a
desire to help others," said Dr. Richard Davey, chief medical officer for
the American Red Cross in Washington, D.C.
As a result of concerns about blood received from people with
hemochromatosis, millions of pints of healthy blood go into hazardous waste
bins around the country, doctors say.
The extent of the disease
Hemochromatosis is a genetically-inherited disease that affects as many as
32 million Americans. Most don't even know they have it.
The disease often masks itself with symptoms of other diseases. Chronic
fatigue syndrome and diabetes are common manifestations of hemochromatosis.
In advanced states, the heart, liver, pancreas and reproductive system can
be damaged, doctors say.
The excess iron literally destroys the organs over time if blood is not
taken from the patient.
"If you have hemochromatosis or iron overload, a portion of those people
will have some hepatitis as a result of iron accumulation in the liver.
Some will have risky lifestyles. Both portions will not be able to donate
blood," said Dr. Geoff Block, a liver specialist and director of the
Hemochromatosis Center at the University of Pittsburgh.
But that still leaves a portion of those with hemochromatosis who has no
lifestyle or medical reasons for not giving blood, he said.
"That portion of people with hemochromatosis should be able to donate
blood. It should be available for transfusion. There is nothing abnormal
about that blood or that donor," Block said.
Countries outside the United States have been taking the blood of
hemochromatosis patients for years and using it for transfusions, Block said.
In 1991, Canada started using iron-overloaded blood, he said.
"Its been going on in Canada for years, and we know people aren't dying
from the blood. If Canadians can survive, we probably can too," he said.
In 1996, the gene that causes hemochromatosis was discovered.
"It's become evident that nothing is going to be transmitted in the blood
for people who have hemochromatosis," Block said.
In fact, the blood that is rich in iron might even benefit more patients,
said Dr. Victor Herbert, chief of the hematology research lab at Mount
Sinai School of Medicine in New York.
Many patients needing transfusions are low on iron, Herbert said.
Each year, blood banks around the country send out urgent pleas for more
blood donors.
This time of year is particularly bad because blood banks must compete with
Christmas activities for blood donors' time, said Kiss of the Central Blood
Increases in traffic accidents and organ transplants also deplete the blood
supply, he said.
A matter of labeling
One of the problems that must be overcome before blood drawn from
hemochromatosis patients can be used has to do with the way it is labeled.
The Food and Drug Administration requires that blood donated for
therapeutic purposes - such as the blood from hemochromatosis patients - be
labeled conspicuously to indicate the donor's disease.
"We have found in the past, that doctors and patients are reluctant to use
blood with a donor's disease labeled on it. It tends to sit on a shelf,"
said Davey of the American Red Cross.
But Herbert has tried to change that perception.
In 1996, he petitioned the FDA to change the labeling process for
hemochromatosis donors. In his request, he asked the label be changed to
"volunteer donor."
Herbert based his request on the fact that hemochromatosis cannot be
transmitted through transfusion.
It is probably a safer alternative to the donor who walks in off the
street, Herbert said.
"All patients with hemochromatosis are under a doctor's care and have
regular blood drawings to check their blood for everything," he said. "They
are checked for things that the blood banks don't do in their routine
Blood banks check for Hepatitis B and C and the AIDS virus.
The FDA, however, denied Herbert's request, claiming there was not enough
evidence to support his position.
But he isn't giving up.
In November, he spoke at the annual meeting of the American Association of
Blood Banks and found some support from the audience.
He said he is hoping to see some changes in the use and labeling of
hemochromatosis blood in the next few months.
Other groups also are looking at the situation.
The American Medical Association is studying the use of blood taken from
hemochromatosis patients and its possible use in the blood supply. The
medical organization could take a stance on the use of hemochromatosis
blood by July 1999, a spokesman said.