Prune Belly Syndrome | Powerful Patient

From Mike Lawing:

The Exhibit Hall in the San Diego Convention Center is huge. It covers an area equal to nine or ten football fields. Recently when the American Urological Association held its annual meeting the 18,000 or so attendees streamed through the exhibit hall looking at the latest robotic surgical devices and various tools of the medical trade designed to enable doctors to peer inside bodies of their patients to find kidney stones and a host of other maladies that affect them. In addition to the gadgetry of the 21st century, companies presented their medical concoctions developed with great effort (and cost) designed to restore health, vitality, and a normal life to those who suffer ailments that a few decades ago often proved to be debilitating or fatal. Elaborate displays, hands on events, and knowledgeable highly trained representatives of supply companies were there to arrange for a test-drive of their equipment on site in hospitals and clinics around the world. The annual AUA meeting draws crowds of urologists and other specialists to hear of the latest medical advancements in seminars and sessions and to mill through the exhibit hall. Tucked away in secluded corners of the massive technology- filled room was smaller, almost inconspicuous booths of the nonprofits. These exhibits lacked the plush carpets, huge expanses of interactive displays, and well-paid scores of representatives eager to market the latest innovations. Most of these displays consisted of a table, a few chairs, some literature about the organization, and occupied 100 square feet of the half-million square feet Exhibit Hall. It is within the confines of a 100 square foot exhibit staffed by four remarkable people that our story takes place.

Derek hails from a small town in South Georgia just north of the Florida line. He serves as a volunteer youth ambassador for the PBS Network, a small nonprofit that addresses a health issue that few have heard of. In many respects this youthful 22-year-old man with his incredibly warm personality and handsomeness that would make him a leading candidate for a movie or TV program (such as a remake of The Dukes of Hazzard) is seemingly out of place in this obscure exhibit. He should be elsewhere in the exhibit hall selling those million dollar medical devices; he is articulate and loaded with charm and the qualities that super sales reps covet.

Maryanne, Debi, and Loretta are from Pennsylvania, New Jersey, and Ohio respectively. This is Maryanne’s first time at a convention of this sort; it is also her first time away from her children for any length of time since they were born. She serves as the President of PBSN, Debbie is a vice president, and Loretta is treasurer. Their exhibit has the usual brochures, business cards, and information about what they so passionately do. There is also a seemingly bottomless bowl of Hershey’s Miniatures, wristbands with the organization’s website and their slogan “Support for today… Hope for tomorrow,” and silver ink pens emblazoned with contact information for an organization with a curious name: Prune Belly Syndrome Network, Inc.

The exhibit with a curious name also has photos — many photos; photos of families with young children, with carefree smiling faces. There’s a photo of Derek from his high school days in his ball uniform as he swings for the bleachers; there are also a number of photo portraits on canvas of cute babies and young children with a sparkle in their eyes and a smile on their face — and then the eye of the observer is drawn from the smile and the eyes to the belly. It is a belly that is strangely out of place on such a beautiful child; a misshapen, wrinkled, prune-like form that somehow should not be. It is something called Eagle-Barrett Syndrome, or Prune Belly. It occurs only once in about 40,000 births, 95% of those with prune belly are boys. With only 100 cases or so of new births in the United States each year, it is a very rare condition. Many of the urologists, nephrologists, and other professionals who are filing through the exhibit hall that stop by the prune belly exhibit have never seen it in person. Perhaps they remember a paragraph-sized reference from med school; some have had one, perhaps two cases in their years of practice –rarely more than that. It takes courage for doctors to admit their lack of knowledge on a medical condition, but the photos of those with prune belly are so shocking that somehow it lowers many of their inhibitions. The reality of prune belly is that there is currently no existing standard of care; many doctors who see indications of the syndrome in the fetus will encourage abortion, citing possibilities of numerous health issues, and a high mortality rate before the age of two. It is not uncommon for doctors who have an elementary level of understanding about prune belly to be shocked when they learn that Derek was born with PBS; living proof that survivorship into manhood is possible. The condition is so uncommon that Derek lived for 20 years without seeing another person with PBS. Maryanne, Debi, and Loretta all have children with PBS; they work hard as volunteers with other members of the PBS network to raise money for an annual meeting of those with PBS as well as their families. Additionally they are striving to raise awareness of the condition. Their children who have PBS require extra attention and a very high level of care. Some parents who have PBS children were told not only by their family doctors and pediatricians but by numerous specialists to have an abortion. The parents who chose not to follow that conventional wisdom report that they don’t regret their choice.

The Prune Belly Syndrome Network faces tremendous challenges as they work to raise awareness of this rare condition; as Powerful Patients, we can assist them by spreading the information to those we contact by e-mail and other means. Someone in your community may be carrying a fetus or have a child with this very rare syndrome.

More information is available at: Prune Belly Syndrome Network, Inc.

P.O. Box 16071, Philadelphia, PA 19154