Families Burdened by Hemophilia Turn to State

By DEBI AKCHIN GRAHAM
Special to the Advocate

*Published in The Advocate on July 19, 2004

Advocate staff photo by Richard Alan Hannon
Tristan Conway's parents, Shelby and Connie, keep a constant eye on their son who suffers from hemophilia.

As 4-year-old Tristan Conway climbs several stairs, jumps down and swings from a stair railing, his parents, Donnie and Shelby Conway of Paulina, watch his every move.

They try to sound casual as they warn the rambunctious boy to be careful, while never taking their eyes off their only child.

Allowing their son the freedom to live a normal life is a daily struggle, Shelby Conway said. Every bump or cut is potentially life-threatening because Tristan's body does not produce the material needed for his blood to clot.

Injuries are just one of the fears faced by families living with hemophilia. How to pay for treatments is another huge concern.

Hemophilia, which is not contagious, is an inherited disease. Women are the carriers, but except in extremely rare cases, hemophilia is found only in males.

The disease occurs in 1 of 7,000 male births. About 10,000 people in the United States are afflicted with hemophilia, officials said.

Treatment requires frequent infusions of a special blood-clotting product known as factor. The amount needed depends on a person's weight, but each unit of factor costs about $1.

For several months, Tristan received 1,100 units of factor a day; later, he was getting 2,200 units a day.

Shelby Conway estimated their expenses for one year of the immune tolerance therapy -- one of the two therapies they've tried for Tristan -- were about $600,000, paid primarily through their health insurance.

Shelby Conway, 29, is the assistant benefits director for LSU's Human Resources Department. Donnie Conway, 35, is an instrument and electrical technician for Honeywell Specialty Chemicals in Geismar.

If a family begins preventative treatment for a child at age 1, their costs for factor will probably exceed $1 million by the time the child is 12, said Lori Keels, director of the Louisiana Hemophilia Foundation.

"It is not unusual for our families to have to quit a job and find another position with better benefits because they have maxed out the lifetime cap on their health benefits," Keels said.

Louisiana is one of 31 states with factor programs. Whether patients have insurance, Medicaid, or no medical coverage, they can receive factor through the Louisiana program in New Orleans. Reimbursements from patients with insurance help cover the cost of factor provided to people without coverage.

Karen Wulff of the Louisiana Hemophilia Treatment Center at Tulane University School of Medicine said the state funding has been threatened in the past, but the hemophilia community has actively lobbied to retain it.

The treatment center at Tulane was established in 1980 by the state and the U.S. Bureau of Maternal Child Health. Additional funding comes from the U.S. Centers for Disease Control and Prevention.

Keels said another fear faced by people with hemophilia is the safety of the blood factor products.

"There is always that fear -- that possibility -- that the blood factor products they must use to survive could contain some new virus no one knows about yet."

Many hemophiliacs treated before 1990 were infected with the Hepatitis C virus, which can cause serious damage to the liver and which was in much of the blood supply for several years before a test was developed to isolate it.

"The pharmaceutical companies tell us that the heating processes used for their newest blood products mean that there is almost no chance of a virus," Keels said. "But the operative word is 'almost.'

"There is always the fear of a new virus that the pharmaceutical companies don't have a test for. Last year, there was SARS; before that was Hepatitis C. Many hemophiliacs have died from AIDS because of blood products they received before the test for HIV was developed," Keels said.

Donnie Conway said their biggest fear is the possibility of Tristan suffering a head injury. Donnie Conway installed carpeting throughout their home and said they try to monitor Tristan's activities as much as possible. On at least three occasions, he said, they rushed Tristan to the hospital for a CAT scan after the boy bumped his head.

Tristan, however, played with abandon when the family attended a hemophilia conference recently in Baton Rouge.

"Hemophilia is always going to be a guessing game, especially until Tristan is old enough to really tell us how he feels," Donnie Conway said.

"We try not to shelter him too much," Shelby Conway said, "but he knows we're going to react with fear every time he falls or bumps something. Usually when he falls, he jumps up immediately and tells us, 'I'm OK!'"

On the Internet:

Louisiana Hemophilia Foundation (800) 749-1680

http://www.louisianahemophilia.org/

*Published in The Advocate on July 19, 2004