He lacks immune system, but local boy full of life

He lacks immune system, but local boy full of life
From the Wisconsin State Journal
Susan Lampert Smith ssmith@madison.com
Contact Susan Lampert Smith at ssmith@madison.com or 608-252-6121.
COTTAGE GROVE – Josh Osborn was born with the same scary condition as “the boy in the bubble,” but 7-year- old Josh is definitely not living his life in a bubble.

He’s the boy jumping off the coffee table.

He’s the boy climbing the back of the couch to capture his very patient cats, Pumpkin and Esther.

He’s the boy in the second grade at Taylor Prairie Elementary School in Cottage Grove, where he gets good grades and has a bunch of friends.

Most importantly, he’s a kid who is full of life as he waits for a gene therapy trial that holds promise for curing his condition, which is called severe combined immunodeficiency (SCID), and means that Josh was born without a working immune system.

His parents, Clark and Julie Osborn, are thrilled Wisconsin is the first state to study whether to test all newborns for the condition.

“This is what all SCID kids could look like,” Clark Osborn said, as he watched Josh chase the cats. In fact, he added, they might look “even better, because he hasn’t been cured yet.”

Science has advanced considerably since David Vetter was born in Houston in 1971 and whisked immediately into a sterile bubble, where he spent 14 years waiting for a cure before he died. (Vetter’s older brother died of SCID in infancy.)

Today, babies with SCID who are diagnosed early and have a bone marrow transplant before age 4 months have more than a 95 percent chance of being cured. Donated bone marrow allows them to grow a new immune system.

The problem is that most babies aren’t diagnosed until they have suffered through a string of serious illnesses. Experts say SCID is the cause of many unexplained infant deaths. And the success rate of transplants falls off dramatically as the babies get older.

The Jeffrey Modell Foundation, which is launching the pilot screening with the state Laboratory of Hygiene at UW-Madison and Children’s Hospital of Wisconsin in Milwaukee, estimates that testing 70,000 Wisconsin newborns each year will find one child with the condition.

You could say that it was luck Josh Osborn’s SCID was discovered before he became seriously ill.

But that’s not how his parents see it.

“We believe it was divine intervention,” Clark Osborn said, “but people who don’t agree with that would say it was luck.”

Neither parent has a family history of the inherited disease. But when their 2-month-old son developed a strange lump in his groin, they took him to Urgent Care, where a doctor ordered a blood test. It showed Josh had no white blood cells. A second test confirmed those results.

Medical science advanced since the “bubble boy” era and so has the Internet.

Clark Osborn recalls that Josh was diagnosed on a Friday, and within hours, he was in e-mail contact with Duke University’s Dr. Rebecca Buckley, a leading expert on the disease. Within days, Josh had an appointment with Buckley, and his parents had joined an Internet support group for the disease.

Julie Osborn and Josh moved to North Carolina for several months, and at age 5 months, Josh had a “half- match” bone marrow transplant from his mother. The transplant fixed his immune system for more than two years, until it began to fail.

Since age 3, he’s been on a weekly injection of an immune-boosting drug that costs $3,200 per vial. Once a month, he gets an infusion of another drug.

Other than that – and the fact he stays home when chicken pox is going around school – Josh has been a healthy and happy boy.

His parents say that the Dean Health Plan has been great about covering his medicine and referring him to Duke. But they hope for a permanent cure from a gene therapy experiment set to begin soon at the National Institutes for Health.

They hope the pilot study will become permanent, so that if a baby is born with SCID in Wisconsin, the child will have as good or better a chance at a healthy, relatively normal life as Josh.

Finding that one child a year is a matter of life and death, the difference between life in a bubble and a life full of life.

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