Treatment for a Nampa baby with a rare and dangerous condition raises complex issues for doctors, parents, insurer

By Colleen Lamay

February 3, 2009

Heather Danti holds her son, Lucca, who was born on Dec. 9th with DiGeorge Syndrome, a rare congenital abnormality, in the St. Luke's neonatal intensive care unit in Boise. On Monday Lucca will be flown to the Duke University Medical Center in North Carolina where he will undergo treatment which will include multiple surgeries. Joe Jaszewski / Idaho Statesman

Tiny Lucca Danti lies in a crib at St. Luke's Boise with a rare condition that will kill him unless he gets at least two surgeries and care that likely will total more than $1 million.

The treatment is no guarantee the 2-month-old, who will likely have hearing and vision problems regardless of treatment, will live a relatively normal life, but it may be his best chance.

The frail child's case exemplifies everything that is right, wrong and heartbreaking about our health care system.

"It's exhausting," father Ed Danti, of Nampa, said of the hurry-up-and-wait of slogging through health insurance procedures and paperwork to see if Blue Cross of Idaho will cover a transplant the parents and doctors want but that many insurers still consider experimental.

"The bottom line is the front troops are sympathetic, and they are trying, but they have to go through this chain of command to get a straight answer to a simple question," Danti said.

Lucca was born at St. Luke's Boise Medical Center on Dec. 9 with what appears to be the most severe, and least common, form of DiGeorge syndrome, a condition that is rare in any form. Symptoms can vary greatly, but generally a baby's immune system suffers a huge blow, making a common cold a potentially life-threatening illness. Heart defects also may be present. Lucca has those, too.

"It's really an issue in how our health care system is structured," said Dr. Declan O'Riordan, Lucca's neonatologist at St. Luke's Boise.

"We are structured to have insurance companies deal with usual conditions," he said. Rare conditions are sometimes so rare there is not enough evidence to back one procedure over another in a definitive way. "We as a society have to decide how we are going to deal with that."

Lucca and his mother are expected to take a special medical flight Tuesday or Wednesday to Duke University Hospital in Durham, N.C. There, he will undergo one or more extremely complicated heart surgeries, which Blue Cross of Idaho already has said it will cover.

What will happen next is undecided. His family and doctors in Boise and at Duke think a thymus transplant might be a cure for Lucca. Blue Cross is holding back, waiting for the results of more tests.

The insurer and Duke itself label the transplant of a thymus - a small organ in the upper/front portion of the chest - experimental, while a bone marrow transplant for the same condition is not. If the insurer denies the thymus transplant, it would cover a bone marrow transplant.

As it gets better at treating ever more complex medical conditions such as Lucca's, the cost of health care takes a bigger bite, raising health insurance premiums and leaving more people unable to pay their bills.

"It's not hard to understand," Blue Cross spokeswoman Karen Early said. "If there are a lot of cases that are very high cost, ultimately that is going to affect everyone's premiums."

Blue Cross of Idaho, which is the insurance Ed Danti gets through his job in construction, is seeing more $1 million cases, as are insurers nationwide. Lucca will likely use up his $1 million Blue Cross lifetime benefit before he is 1 year old.

Despite the cost, no one is willing to say an infant should go without treatment. "We try very, very hard to meet the needs of our patients," Early said.

Lucca's family - Ed; his mother, Heather; brother, Jackson, 2; and half-siblings Madyson, 10; Ivy, 10; Ethan, 7; and Taylor, 4 - get more frustrated as time passes. Waiting means a risk of complications from infection or heart trouble.

"Part of me, when they were arguing over the medical insurance, I was thinking, 'Just get him there, and I don't care how it gets paid,' " Ed Danti said. "We deal with it by going, 'Hey, what else can we do?' "

Two months in the neonatal intensive care unit at St. Luke's Boise already has cost about $200,000, a Blue Cross spokeswoman and Ed Danti said.

The arguments back and forth, the pros and cons of a thymus transplant vs. a bone-marrow transplant, rely to some extent on the outcome of tests that have yet to be conducted.

Determining the very best treatments for extremely rare diseases takes time. The number of patients is tiny, and data based on small numbers of patients is notoriously slippery.

Bone marrow transplants are a more common procedure performed for a variety of conditions at many medical facilities. Some data is available on bone marrow transplants for DiGeorge syndrome, but the number of patients in the studies is very small.

So far, Lucca looks like a very good candidate for a thymus transplant, said Dr. Louise Markert, the immunologist who has pioneered thymus transplants at Duke.

Much will depend on how the infant weathers his heart repairs. And there is no substitute for Markert in seeing the baby and examining him when he gets to Duke.

"I look forward to meeting Lucca and holding him in my arms and looking in his eyes," Markert said. "Every baby is wonderful."

Colleen LaMay: 377-6448

How To Help

A fund has been set up at Home Federal Bank to help the Danti family defray the ongoing expenses associated with Lucca's care. Call or go to any branch to donate to the "Love 4 Lucca" account.

More About Digeorge Syndrome

The chance that a baby will be born with DiGeorge syndrome is about one in 3,000 to 5,000.

One of 500,000 newborns will have as severe a case of DiGeorge syndrome as Lucca.

Dr. Louise Markert, the Duke Hospital immunologist who would perform Lucca's surgery, has been the subject of mainstream media coverage that describes thymus transplants as a cure for some patients.

The thymus is a vital gland behind the breastbone that guides immature white blood cells from the bone marrow so they can develop into T-cells that fight off infection.

Duke is the best choice for Lucca, doctors, parents and the insurance company agree, because Duke is the only hospital in the United States that can repair Lucca's heart and also has a track record performing thymus transplants.

Markert performed her first thymus transplant more than a decade ago. Today, that baby is a healthy, normal 15-year-old, Markert said.

After 58 transplants, Markert's procedure now boosts a 74 percent success rate, Markert said.

Sources: Dr. Louise Markert, professor of pediatrics and director of the Laboratory of Thymus Transplantation at Duke University School of Medicine; Dr. Declan O'Riordan, neonatologist at St. Luke's Boise Medical Center; Duke University School of Medicine Web site.

Copyright © 2009 IdahoStatesman.com.

This article posted March 29, 2009.