Chance Of A Lifetime
August 05, 2001

By Nancy McVicar Health Writer

Using a technique never before performed on a child so young, doctors at Children's Hospital Boston on Tuesday gave 14-month-old Spencer Crawford, dying from a condition called pulmonary vein stenosis, one more chance at life.

Spencer, of Boynton Beach, has survived open-heart surgery at 3 days old, a heart transplant at 3 months, and two other surgeries to improve blood flow, including insertion of stents -- tube-shaped devices to prop open his diseased pulmonary veins.

Tuesday's six-hour procedure, similar to cardiac catheterization in adults with clogged heart arteries, cleared an overgrowth of tissue from two stents in his right pulmonary veins.

Tiny radioactive beads were threaded into each stent and left in place for 41/2 minutes to irradiate the remaining tissue and vein. The hope is that it will prevent the clogging tissue, which could suffocate Spencer, from growing back.

"The reason we are trying the [radiation therapy] is that it has worked in adults," said Dr. Stanton Perry, chief of invasive cardiology at the hospital and associate professor at Harvard Medical School.

Spencer is scheduled to get weekly chemotherapy aimed at halting the progress of the disease in the veins outside the stents. After the treatment in Boston, he will return home and continue the chemotherapy in South Florida.

Pulmonary vein stenosis is a rare condition in which the lungs' veins become constricted or blocked, preventing freshly oxygenated blood from flowing into the heart and being pumped into the rest of the body.

Normally, arteries carry oxygen-rich blood, and veins carry oxygen-poor blood, but in the pulmonary circulation, their roles are reversed.

The cause of the disease, which has a high mortality rate, is unknown. Some children who have had heart procedures develop it, but so do others who have never had cardiac surgery.

Fragile procedure

A team led by Perry had the delicate task of threading a tiny catheter through Spencer's hepatic vein, the major vein in his liver, through his heart and into the right pulmonary veins.

"Technically, it was quite, quite difficult, because the patient is extremely small," said Dr. Phillip Devlin, of nearby Brigham and Women's Hospital. The doctors had to maneuver the catheter, which can be a little stiff, around a sharp left angle to reach the stents.

Perry used a balloon procedure to push back unwanted tissue threatening to clog the stents. Then Devlin, director of brachytherapy at Brigham and Women's, threaded in the tiny radioactive beads. The beads emit beta radiation, which travels only a short distance.

"There were eight hands all pushing or pulling something to make that turn and get [the beads] in and to keep them there during the therapy," Devlin said. "It was a real tour de force. I'm very proud of the work. The child was given zero hope, and I think we've given some hope."

The dosage, confined just to the stent and the blood vessel, was calculated by Devlin and a physicist, based on the size of the tiny areas to be irradiated. Each stent is about an inch long, and the radiation extended into the margins on either end to suppress cell growth outside the stents as well.

The doctors were unable to perform a similar procedure in his left lung, because a stent on that side had dislodged and moved out of the vein, which was too constricted to be reopened.

Because the left lung is not functioning, the right must supply all the oxygen his body needs, but Perry told his parents he could survive with only one working lung.

The same team performed the brachytherapy procedure five weeks ago on Lindsey Cox, of Mobile, Ala., who turned 4 on Monday. She is still a patient at Children's, and it is too early to know whether the technique worked.

Spencer will join Lindsey in a clinical trial using chemotherapy in an effort to halt the progression of their pulmonary vein stenosis in areas beyond the stents.

Dr. Kathy Jenkins, associate in cardiology and assistant professor of pediatrics at Harvard Medical School, said an analysis of the cells causing the veins to shut down found they were similar to a type of cancer cell called a desmoid tumor and regarded as an overproliferation of scar tissue.

Jenkins, working with Dr. Mark Kiernan of the Jimmy Fund at nearby Dana Farber Cancer Center, got permission to start the trial using a combination of methotrexate and vinblastine, two chemotherapy drugs given intravenously once a week.

The first patient was enrolled in April 2000. But because the condition is so rare, only about eight patients have had the chemo treatment.

No other options

"The entry criteria is that the physician caring for the child has come to the conclusion there's nothing else," Jenkins said. "There has been some good news and some bad news. Some children have had an arrest of their problem, and others have still had problems. But it's way too small a number to make any definitive statements."

Jenkins said children in the trial are scattered all over the country and get their chemo treatments in their hometowns.

"As chemotherapies go, these are pretty gentle drugs. In general they don't make kids very sick," she said. The side effects can include nausea, suppressed immunity and hair loss, however.

The doctors will continue to monitor Lindsey and Spencer, but it will probably take two to three months before they know whether the disease progression has been halted, Perry said.

In Lindsey, Perry found the blood still flowing about three weeks after the brachytherapy. Her vein had been constricted for an extended period of time, so one of the things doctors want to find out is whether the vein will open up further on its own.

"What Spencer has is very bad. Most kids in his situation die, but we're trying something completely new," Perry said. "There is no guarantee it will work. It's tough for families, because they have this dilemma about whether to put the child through more. But there just isn't anything else."

Nancy McVicar can be reached at nmcvicar@sun-sentinel.com or 954-356-4593.

Publication Date: Sunday, August 5, 2001 Edition: Broward Metro

Section: NATIONAL Page: 1A

Memo: Informational box at end of text. Dateline: BOSTON Publication: SOUTH FLORIDA SUN-SENTINEL

Illustration: PHOTOS 5 Caption: Staff photos/Susan Stocker (color) CARDIAC SURGERY: Doctors at Children's Hospital Boston prepare Spencer Crawford, 14 months, for an experimental procedure to open clogged stents implanted previously to prop open his diseased pulmonary veins. (color) HOPES: Spencer, 12 days after surgery, was 3 months old when he received a heart transplant. RARE CONDITION: Spencer recovers in the cardiac intensive care unit after his brachytherapy on Tuesday. He will receive weekly chemotherapy aimed at halting the progress of the disease in the veins. Without the surgery, Spencer likely would have suffocated as tissue continued to clog stents in his veins. LAST RESORT: Spencer Crawford's parents, Ellen and Chris, speak with anesthsiologist Dr. Alexander Garden before their son undergoes a six-hour procedure to clear overgrowth of tissue from two stents in his right pulmonary veins. AT ONE YEAR OLD: Ellen Kaplan-Crawford learned in June that her son, Spencer, may have only weeks or months to live. Keywords: MEDICAL PROCEDURE CHILD HEALTH

Box: EDITOR'S NOTE In December, the Sun-Sentinel told the story of Spencer Crawford's medical journey to correct hypoplastic left heart syndrome, a defect that left him with half a heart. Now, Spencer faces another battery of operations in an effort to keep him alive.