Wednesday, August 09, 2000

Hemophiliac boy's heart, life restored

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By Tim Bonfield
The Cincinnati Enquirer

        On his last day at Children's Hospital Medical Center, Cortez DeLeon was feeling energetic enough to play air-guitar with a dust mop.

Cortez DeLeon sits with his mother, Yolanda DeLeon (Craig Ruttle photo) | ZOOM |

        Less than three weeks ago, however, doctors weren't at all sure the 7-year-old Dayton, Ohio, boy would leave the hospital alive.

        “We see a lot of complicated cases at Children's Hospital,” said cardiac surgeon Dr. Peter Manning. “But if I see two or three cases like this in a lifetime, I'd be surprised.”

        Saving Cortez was a medical odyssey that took months of planning and weeks of intensive care. His case required special approval from the U.S. Food and Drug Administration and a hospital review board. The work involved an entire team of medical specialists, special monitors and tests, and more than $500,000 worth of products made from pigs, cows and hamsters.

        “Quite frankly, we didn't know if this would work,” Dr. Manning said.

        Cortez needed help because an infection that started about five years ago had eaten away one of his heart valves. As a result, his heart had swelled to nearly three times its normal size.

        Without open heart surgery, Cortez would die.

SAVING CORTEZ

Saving Cortez DeLeon required extra effort on several fronts:   • Using an experimental replacement heart valve, made from a cow, required “compassionate use” approval from the U.S. Food and Drug Administration, plus a review by the Children's Hospital Institutional Review Board.   • During the procedure, Cortez needed special monitoring equipment, extra lab tests and care from a surgical team expanded to include experts in hemophilia.   • While recovering, Cortez needed large amounts of two special types of blood-clotting factors. Combined, the products cost more than $500,000.   • Rather than going home within four or five days after surgery, Cortez needed to stay more than two weeks at Children's Hospital Medical Center, mostly in intensive care.

        But there was a problem: Cortez was born with a rare type of hemophilia that not only prevents healing from wounds but also destroys the most common blood-clotting products that help most other hemophiliacs.

        With all the cutting required for open heart surgery, that was a problem.

        “It was a really scary thing, but it was something that had to be done,” said Cortez's mother, Yolanda DeLeon. “We spent 2¹/₂ years trying to decide all the hows, whens and wheres. God only knows how much longer he had.”

        About one in 10,000 Americans, mostly boys, are born with hemophilia. The condition means their blood lacks the normal ability to form clots, said Dr. Ralph Gruppo, director of the Children's Hospital hemophiliac treatment center.

        But Cortez has a condition found only in about 10 percent to 15 percent of all hemophiliacs. His body produces antibodies to the blood-clotting factors intended to help him heal, making wound treatment very difficult, Dr. Gruppo said.

        Knowing that surgery would be a life-threatening risk, Mrs. DeLeon and the doctors at Children's Hospital waited until his deteriorating health forced them to act.

        For Cortez, even the type of valve to be used required special effort.

        Artificial valves require follow-up care with blood-thinning medications that would be bad for Cortez. Doctors could try valves made from pig tissue, which don't require blood thinners, but they last only about five years in children, Dr. Manning said.

        A possible solution emerged when Dr. Manning learned of a longer-lasting heart valve in Europe made from cow tissue. The device had been used in Europe for about 15 years. Trouble was, the product had not been approved for use in the United States for the type of valve Cortez needed to have replaced.

        Dr. Manning and the manufacturer, Edwards Labs, pushed the paperwork needed to win “compassionate use” approval from the U.S. Food and Drug Administration.

        They also needed and got approval from the Children's Hospital Institutional Review Board.

        Beyond the valve, a plan was needed to help Cortez recover from the surgery. Even with sutures to close the wounds, every person who has open heart sur gery bleeds at least a little until the body's natural clotting ability closes off tiny, severed blood vessels and gaps between the stitches.

        This is where Dr. Gruppo came in.

        The plan for Cortez called for using massive amounts of two blood-clotting products.

        First, doctors would try a porcine version of human clotting factor VIII. The hope was that the animal product would be different enough to bypass Cortez's resistance to the human product.

        As a second step, doctors would use a new product called NovoSeven, made by genetically modifying hamster ovary cells to produce human clotting factor VII.

        “Even the factor seven product is not 100 percent effective,” Dr. Gruppo said. “Sometimes it doesn't work at all.”

        Doctors couldn't tell Mrs. DeLeon whether Cortez had a 90 percent chance or a 10 percent chance.

        “We really couldn't define the risk. That was one of the reasons we delayed the surgery until it was absolutely necessary,” Dr. Gruppo said.

        With approvals from Mrs. Cortez, the government and the hospital in hand, Cortez was admitted to Children's Hospital on July 18 with surgery set for July 19. The surgery lasted about four hours.

        During the procedure, Cortez needed special monitoring equipment and lab tests to track the levels of his blood clotting factor.

        Even after surgery, Cortez was kept heavily sedated at first while hospital staff pumped him with blood-clotting factors and tested his blood nearly hourly.

        After a week, small internal bleeding had built up to about a quart of blood filling the sac surrounding Cortez's heart.

        An emergency surgery to drain the fluid was required. Fortunately, the clotting levels generated by the NovoSeven were enough to help Cortez handle reopened wounds.

        “After that, he has done very well,” Dr. Gruppo said.

        By Monday, Cortez was headed home to Dayton, where he plans to be back in school in September.

        “He's an amazing kid,” Mrs. Cortez said.

       

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