By Liana Jacob
RARE CONDITION has left this toddler with a PROTRUDING BELLY so big it’s CRUSHED one of his kidneys.
Stay-at-home mum, Violet Smith (33) from Los Angeles, California, USA, and her partner, Lionel (43), had always wanted children since they started dating six years ago.
But their plans were derailed when she was diagnosed with stage four endometriosis in July 2016, a condition where tissue similar to the lining of the womb starts to grow in other places, such as the ovaries and fallopian tubes.
She had to undergo two back-to-back surgeries to remove large grapefruit-sized endometriomas (or growths), and to separate her lower abdominal organs that had been fused together with scar tissue. The disease was destroying her reproductive system. At the time, Violet and her partner wanted to get married, but when her doctors told her that they had to remove her ovaries to prevent the disease from spreading, they had to act fast.
They put their wedding on hold and agreed to try for a baby before her ovaries were due to be removed in the summer of 2017. To their surprise and delight, Violet discovered she was pregnant in July 2017 and gave birth to their daughter, Emelia (now 2). Nine months later Violet and Lionel were shocked and excited to find out she was pregnant again.
When Violet was 16 weeks pregnant, they went for a routine ultrasound scan where they were meant to find out the sex of their unborn baby. However, on the screen, all she could see was a black hole in her womb. She was terrified there was something very wrong.
It turned out that the ‘black hole’ was their son, Oliver’s, who is now six months old, bladder that had enlarged so much it had taken over the screen. Oliver’s urethra, a tube which connects the urinary bladder to the urinary meatus for the removal of urine from the body, didn’t form properly due to his condition. This caused his urine to back up, which expanded the size of his bladder until it was bigger than his whole body.
Although he wouldn’t be diagnosed until after birth, Oliver had Eagle-Barrett syndrome, also known as Prune Belly, a rare condition characterised by partial or complete absence of the stomach muscles, failure of both testes to descend into the scrotum and/or urinary tract malformation.
Before he was born, Oliver had to have a life-saving foetal surgery, where they placed two shunts to drain his bladder and restore his amniotic fluid. Unfortunately, he had to go through four excruciating weeks of foetal testing to rule out genetic abnormalities before qualifying for the surgery, all the while being crushed by his own bladder.
While the surgery was successful, there was significant damage to his kidneys and as a result, Oliver was in complete kidney failure when he was born. He was put on dialysis at just one-week-old. He defied the odds by surviving through as the mortality rate of dialysis in infants is very high at 40 per cent.
When his one remaining kidney miraculously regained some function, Oliver began urinating normally and was spared dialysis treatment. However he was diagnosed with chronic kidney disease which means he will need a kidney transplant once he hits the weight requirement, typically by the age of three to five. Violet and Lionel were distraught by the news and are hoping they will be a match so that one of them can be his donor.
Despite the ordeal he’s been through at such a young age, Oliver has continued to brighten the day of strangers with his cheeky smile.
“Our family’s story may not be typical, but it’s a beautiful story none-the-less. Ours is a story of the strength love provides. A story of rising above, beating the odds, surviving, fighting,” Violet said.
“Lionel and I had been dating for several years and were excited to get married and eventually start a family together. Our plans got a little derailed when I was diagnosed with stage four endometriosis.
“The disease was destroying my reproductive system and starting to attack other parts of my body. When my doctors wanted to remove my ovaries in an attempt to stop the disease from spreading, Lionel and I made the decision to put everything else on hold and try to get pregnant while we still could.
“Our dream wedding was going to have to wait; having a family was important to us and we didn’t want to lose our opportunity.
“We were successful. Fortunately, we were able to get pregnant very quickly with our daughter, Emelia. The hormones stunted the endometriosis, but the pregnancy was hard on my body. I had hyperemesis gravidarum and vomited every day until birth.
“Our doctors quickly recommended having another baby right away to give the endometriosis a good ‘one-two punch’ and also because this might be our only chance for another. It didn’t take us long to decide we wanted to go for it.
“Shortly before our daughter turned one, we found out we were pregnant again. We were elated. Many women with stage four endometriosis are unable to get pregnant at all, and we were fortunate enough to get pregnant twice.
“We gave up on the dream of eventually having a wedding and exchanged tearful, heartfelt vows to each other in front of our immediate family.
“Things may not have happened how we had planned, but we were building our own little family and we were so happy.
“When it came time to find out the sex of the baby, we showed up to the appointment glowing with joy for our growing family.
“The doctor began the ultrasound and my stomach immediately sunk. There was no baby in sight, only a big black hole.
“Something was very wrong; fear washed over us as the doctor tried to make sense of what we were looking at.
“The big black hole was Oliver’s bladder; it was so enlarged you couldn’t see anything else. We were referred to a foetal surgeon, who told us the baby needed an emergency life-saving surgery if he was going to survive.”
Violet and Lionel were told that before they could attempt surgery, Oliver needed to undergo a series of tests to check for any genetic abnormalities. The doctor stuck a giant needle through her belly to extract some amniotic fluid and then performed a similar procedure to harvest some of Oliver’s cord blood.
Luckily, four weeks later the tests came back normal and he was ready for surgery. Their family have set up a Go Fund Me page to help raise awareness of Oliver’s story and hopefully donations to help with any future surgeries.
“It was the scariest thing I’ve ever done. I couldn’t be put under anaesthesia for the safety of the baby, so I tried to lay as still as possible even though I could feel them cutting through me,” she said.
“In fact, the entire pregnancy was scary. I battled depression, walking around in a fog wondering if my baby boy was going to survive. I tried to put my energy towards my then-one-and-a-half-year-old daughter; I knew she needed me.
“Oliver’s urethra didn’t form properly, causing his urine to back up until his bladder was bigger than his whole body while in utero.
“His distended bladder was crushing him, causing kidney damage, prohibiting his lungs from growing properly, and making it so he was unable to build abdominal muscles.
“He had to have a life-saving foetal surgery before he was born during which they placed two shunts to drain his bladder and restore his amniotic fluid.
“The surgery was a success, but significant damage had already been done; Oliver was born with complete kidney failure and without abdominal muscles to keep his organs safe and help his body function like it should.
“He had to go on dialysis at one week old and we were devastated because the mortality rate for infants on dialysis is very high.
“After a few weeks of dialysis, Oliver’s dialysis catheter started leaking and the treatment was put on hold. Then something miraculous happened.
“Oliver’s one kidney he had left (the other had been nearly entirely reabsorbed by his body) kicked on and he started making urine.
“He was able to come off of dialysis and his kidney function is managed with medications. He has chronic kidney disease and is in and out of complete kidney failure because his kidney is so very sensitive.
“He will need a kidney transplant but will not meet the weight requirement until around three to five years old. I’m hoping I will be a match so I can be his donor, and Lionel hopes so too.
“He also suffers from decreased mobility and developmental setbacks because of his lack of abdominal muscles and precarious medical condition.
“He receives physical therapy twice per week and occupational therapy to work on his ability to eat by mouth once per week.
“Oliver is the sweetest baby you’ll ever meet. He’s always smiling and thinks his big sis is incredibly funny no matter what she’s doing.
“He has spent a majority of his life in the hospital and has had five surgeries to date with many more to come.
“The nurses at his hospital joke that nobody bothered to tell him he’s sick. We’re so proud of him and hope to encourage this sweet and carefree attitude throughout his life dispute the difficulties he will face.
“He is going to face a lifetime of medical needs, but we’re prepared to face whatever challenges his condition presents.”
For more information on Oliver’s journey, or to donate, please visit: https://www.gofundme.com/f/Oliver-Medina-Medical-Fund