living fully in spite of prune belly syndrome

We found out we were pregnant with Douglas in the spring of 2013, and were excited to be adding to our family. We went for our 19 week ultrasound in July, and were hoping to find out if we would be having a boy or girl. Instead, we found out devasting news. The OB practice where we went had never seen an ultrasound image like ours before --- the baby's belly was so large and full of fluid --- and couldn't give us a diagnosis for our baby.

by sherry habbe

We were heartbroken; we didn't know if that would be the one and only time we would see our baby alive with a heartbeat. I called the St. Louis Fetal Care Institue (FCI) and made an appointment. Needless to say, I was scared, nervous, and was also hopeful that we would receive a diagnosis and we would be able to save our unborn child. At the Fetal Care Institute (FCI), we met with Dr. Vlastos, who did a thorough ultrasound and gave us our diagnosis --- it was a bladder outlet obstruction, or BOO. It essentially is a blockage in a baby’s bladder that restricts or prevents the flow of urine out of the body, and is also referred to as lower urinary tract obstruction (LUTO). It varies in severity from mild, requiring minimal treatment after birth, to very severe, causing serious kidney problems and requiring surgery. Our son's case was severe and would require surgery if we wanted to give him any chance of survival. Exactly one week after having our first ultrasound, we went into the operating room for fetal surgery. Dr. Vlastos did a Fetal Cystoscopy --- a minimally invasive procedure involving the insertion of a small fiber optic scope into the fetal bladder. This scope is used to remove the fetal obstruction, which improves the odds that the baby will have functional kidneys and lungs, and need less intervention after birth. In addition to the Fetal Cystoscopy, Dr. Vlastos also placed a Vesicoamniotic Shunt. One of the most common fetal procedures, the shunt is a small flexible tube that is placed through the skin and into the baby’s bladder, allowing urine to pass into the amniotic cavity (the bag of water). The shunt can increase the potential for normal function in the fetal kidneys, bladder, lungs, and amniotic cavity. We were lucky that our son did not pull out the shunt after surgery; he was born with it still in place. Not long after surgery, we received a diagnosis of EagleBarrett Syndrome, or more commonly known as Prune Belly Syndrome (PBS). This is a condition characterized by a lack of abdominal muscles, causing the skin on the abdominal area to wrinkle and appear "prune-like", undescended testicles in males, and urinary tract malformations. At one end of the spectrum, the condition may cause severe kidney and pulmonary problems incompatible with life; at the other end, the condition may cause few, if any, urological abnormalities. The cause of PBS is unknown, and treatment varies, including the surgical management of symptoms. Prune Belly Syndrome occurs in 1 in 40,000, and half of those who have the syndrome die by age 2. Douglas was born at 35.5 weeks at SSM St. Mary's and was immediately transfered to the NICU at SSM Cardinal Glennon, where he remained for 84 days. Douglas was born with stage 4 chronic kidney disease, but with multiple surgeries (the first within hours of being born), and medications, he has improved to stage 3 chronic kidney disease, and remains stable. Doctors have determined that only one of his kidneys provide him any function, and his right kidney did not develop properly in utero; Douglas has multi-cystic dysplastic kidneys. Since birth, he has had multiple long and trying surgeries to correct these anomalies, but we are very thankful that Douglas is a thriving 4 year old boy. Despite his condition and all odds, he continues to defy expectations. He continues to grow and amaze us every day. He has taught me what true love is, and what true strength is. Most people never get the chance to meet their superhero; I gave birth to mine.