entire issue [pdf 2.79 mb] - Pitt Med - University of Pittsburgh

COVER STORYA BETTER LIFE FOR THEIR CHILDRENSOMETIMES MEANS TRANSPLANTSPHOTOGRAPHY | CAMI MESATEXT | JOE MIKSCH“PLAIN PEOPLE,”COMPLEX CURESIn a parking lot populated by a dozen or so hundreds-of-horsepower cars and SUVs also sit acouple of one-horsepower conveyances: Amish buggies. This is the Clinic for Special Childrenin Strasburg, Pa. Amish and Mennonite men constructed the clinic here in 1991 and expandedit in 2001.On the front porch, a docile black dog named Bowie greets patients and visitors. Inside are womenwearing unadorned clothes and bonnets. (The Amish, Mennonites, and others who settled the area in the1700s in search of a humble life and religious freedom often call themselves “the Plain People.”) Childrenplay in the waiting room. One child roots through a toy chest full of Beanie Babies. Sunlight streams intothe building from 78 windows. Down a hallway where skylights brighten the oak floor and fir beams (heldtogether with wooden pegs rather than nails, the custom for Amish and Mennonite construction), and pasta genetics lab, a family sits in an alcove in close conversation with physician Kevin Strauss.The family is Mennonite. They are from southern Alabama, not far from the Florida panhandle.Their baby daughter is squirming on her thin father’s lap. The girl has a rare genetic condition calledmaple syrup urine disease (MSUD). It is more common by orders of magnitude among Amish andMennonites—the ratio is one in three-hundred for them and perhaps as high as one in a million forthe population at large, says Strauss. MSUD, which gets its name from the sweet smell of the afflictedchild’s urine, is a metabolic disease that causes amino acids to accumulate in the body. It can lead tobrain swelling, neurological damage, and death. The only cure is liver transplantation. Although MSUDis not a liver problem per se, a new liver compensates for the lack of critical enzymes normally foundthroughout the body. Almost immediately after transplantation, an MSUD patient’s metabolic healthreverts to normal.The Clinic for Special Children, cofounded by West Virginia native and Harvard-trained physicianHolmes Morton and his wife, Caroline Morton, is one of the best places to get diagnosis and treatmentfor genetic disorders particular to the Amish and Mennonites, including MSUD. Children’s Hospital ofPittsburgh of UPMC has become the clinic’s go-to hospital for the curative transplant.Holmes Morton, who cofounded the Clinicfor Special Children (shown left and right)near Lancaster, Pa., now refers all of histransplant patients to Children’s Hospitalof Pittsburgh. In the past three years, Pittsurgeons have performed 38 life-enhancingliver transplants on clinic children.SUMMER 2007 13