“I was 14 years old and doctors were telling me about a lymphatic malformation and saying it wasn’t supposed to be there,” said Jamie Ridener, now 17. She lived in Florida at the time and had undergone a routine test to rule out the possibility of a genetically inherited kidney disease. “They had a problem seeing the left kidney and discovered a cantaloupe-size mass.” In June 2009, surgeons at the Jacksonville hospital removed it and told her to expect the surgical site to drain for awhile. “It never really did,” she said.
Jamie moved from Florida to Clovis, Calif., with her family the summer before her sophomore year of high school. A few months later, her stomach began to swell and her pediatrician referred her to Dr. Michael Allshouse, medical director, pediatric surgery and trauma, Children's Hospital Central California.
“Dr. Allshouse discovered fluid in my abdomen,” said Jamie. “It had been 18 months since my surgery so this seemed to come completely out of nowhere.”
“I had some suspicions of the cause, but when I saw the milky fluid I knew right away what it was,” said Dr. Allshouse.
Jamie’s condition – congenital lymphatic malformation of the retroperitoneum – led to chylous ascites, a rare and challenging clinical condition that allows lymph, the milky-white fluid that circulates throughout the lymphatic system, to accumulate in the abdominal cavity. Dr. Allshouse drained 6 liters from Jamie’s swollen abdomen. He kept an external drain in place after the December 2010 surgery, and Jamie continued losing about 2 liters of lymph daily.
“In retrospect, the mass probably should have been left alone,” said Dr. Allshouse. “The surgery she had in Florida disrupted the area and most likely triggered her problem. Whether it would have started draining on its own at some point down the road is possible, but we’ll never know.”
Jamie began carrying a purse to hide the bag of draining fluid. “It’s uncomfortable when the bag is filling up,” she said. “And draining it at night is painful. It feels kind of like sore muscles.”
Lymph, which contains white blood cells, removes bacteria and certain proteins from the tissues. Lymph also transports fat from the small intestine. The draining left Jamie struggling to gain weight and more susceptible to infection.
“That’s when Dr. Allshouse started doing his research to try and stop the leaking,” said Jamie, whose grandparents did a little research of their own.
“My parents live in Denver and it was hard for them being so far away,” said Debbie Ridener, Jamie’s mom. “My dad made random calls to different hospitals and heard ‘Your granddaughter is in the best hands.’ They told him there was no reason to go anywhere else because Jamie already had the best pediatric surgeon right here at home.”
”Her grandparents were trying to get the best care for her,” said Christine Taylor, Jamie’s aunt. “It gave them such a sense of relief to find out Jamie was in the best possible hands.”
“They say things happen for a reason,” said Jamie about moving to Central California shortly before her medical issues began. “But when you see it working it’s amazing. Dr. Allshouse has been amazing. I can’t even imagine having a different doctor.”
The quest for relief
Faced with a rare condition with no cure, Dr. Allshouse tried a series of treatments and procedures to lessen the flow of fluid from Jamie’s lymphatic system. He prescribed two weeks of total parenteral nutrition (TPN), a type of intravenous feeding, and then another two weeks of TPN and octreotide, a drug that treats gastrointestinal bleeding. Unfortunately, resting the gut made no difference in the amount of drainage.
“I got to eat for one weekend between the two,” said Jamie. “It was heaven.”
Next, Dr. Allshouse performed surgery to locate and seal the leaks, carefully reopening the incision previously used to drain the fluid.
“He said the back of my abdomen was filled with holes like a watering can,” said Jamie. “Dr. Allshouse sealed the bigger holes, but there were too many little leaks everywhere to plug them all.”
A third surgery in April 2011, this time through a longer, vertical incision, revealed holes in Jamie’s intestines as well. “That procedure was a last ditch effort to seal the lymphatic leaks,” said Dr. Allshouse, who continued investigating ways to improve Jamie’s condition. He learned of the Denver shunt, a small tube that redirects lymph from the abdomen to a major artery where the body can absorb it.
“He told us about it and we figured why not give it a try,” said Jamie.
Dr. Allshouse implanted the shunt in August 2011. “For two weeks it worked great,” he said. “Then Jamie called me and said, ‘Dr. Allshouse I have a fever.’ Somehow she had developed pseudomonas, which caused a nasty infection.”
Jamie was admitted to Children’s and Dr. Allshouse removed the shunt. She spent two weeks in the pediatric intensive care unit (PICU) fighting the infection, which had been circulated via the shunt to her heart. “The infection was everywhere,” said Jamie. “My body was septic.”
Pediatric intensivists in the PICU saved Jamie’s life, treating her with intravenously administered antibiotics three times per day. She beat the infection and was discharged.
“Jamie needed IV antibiotics for six weeks, but there was no reason to keep her in the Hospital that long,” said Dr. Allshouse. “We try to get our patients in and out as quickly as we can because children do better at home.”
“I went home with the PICC line,” said Jamie, referring to the peripherally inserted central line used to administer IV medication. “I gave myself antibiotics every day.”
From her PICU stay until January 2012 Jamie stopped leaking lymph. “For three months I had no drain and no abdominal swelling,” she said. “Dr. Allshouse said the inflammation from my infection may have plugged the leaks.” However, as the infection cleared the drainage resumed.
“Then Dr. Allshouse had an idea to try something that’s used for dialysis,” said Jamie.
He surgically implanted a peritoneal dialysis catheter (PD cath). “No one’s ever used it for this purpose before. It was Dr. Allshouse thinking outside the box and it means no more external drain. I love this thing!”
“The PD cath is normally used for putting fluid in and taking fluid out during dialysis,” said Dr. Allshouse. “But Jamie uses it only for draining and it works great.”
Gone was the external drain Jamie had tucked into a purse from December 2010 until August 2012. “The PD cath is so simple it’s amazing,” she said, demonstrating how she exposes the clamped tube, opens the end and drains it into a measuring bag. “It was Dr. Allshouse’s idea to use it and now I’m sure other doctors will be using it, too.”
After finding success with the innovative use of the PD cath, Dr. Allhouse continued to share new research and possible treatments with Jamie.
“Dr. Allshouse was sending my file everywhere looking for ideas to help me,” said Jamie. “He heard about a clinical trial at Boston Children’s where immunosuppressant drugs used for transplant patients were shown to decrease the leaks. We gave it a try but I was draining more on the trial than I had been before.”
“Jamie is the only patient in the trial whose drainage actually increased,” said Dr. Allshouse.
Jamie succumbed to another infection in October, which kept her at Children’s until Thanksgiving. “Obviously it’s no fun what I’m going through,” said Jamie. “But we’ve been so lucky with the people we’ve been able to work with.”
A specialist from Boston Children’s familiar with Jamie’s illness contacted Dr. Allshouse about an experimental procedure on the thoracic duct. “He said I would be the fourth person ever to receive it,” said Jamie. “I said, ‘Let’s give it a try. What do I have to lose?’”
“Jamie’s most positive feature is her indomitable spirit,” said Dr. Allshouse. “She’s bright and has a lot of capabilities, but the single most impressionable thing about Jamie is her attitude.” Unfortunately, the thoracic duct procedure did not affect the amount of daily drainage, which continues to flow at a rate of 2 to 2.5 liters a day.
“My niece doesn’t give up and keeps on plugging through,” said Taylor. “Jamie’s personality inspires others. It is amazing what an attitude can do, and her attitude is that it’s just as easy to be in a good mood as a bad mood so I may as well be in a good mood. We need more people like her in this world.”
“At this point anything they give me is going to be experimental,” said Jamie. “My mindset, obviously, is to look at the risks, but if the doctor says, ‘I think this could help,’ I’ll try it. I know what I can manage and still enjoy my life.”
“My daughter stays so positive and I know that she has to work hard to stay positive,” said Ridener. “We know there’s no cure right now so we’re hoping for longer stretches of good days.” An extended stretch of good days will enable Jamie to attend college in the fall.
“Jamie got a scholarship for college from the Make-A-Wish Foundation,” said Dr. Allshouse. “She was able to tell them her wish and she wanted a scholarship.” He promised not only to keep in contact while Jamie attends college, but also to recommend a specialist if she needs one. “I know who to refer her to because I used to work in Denver. They’re people I would let operate on my family,” he said.
Jamie will graduate from Buchanan High School in May and plans to study psychology at Colorado State University. “I’m thinking about being a psych nurse,” she said. “And I know I want to work with kids.” She shares that interest with her doctor.
“After becoming a general surgeon I took the extra training required to become a pediatric surgeon,” said Dr. Allshouse. “I gravitated to it because of my interest in the needs of kids.” His commitment to helping kids endears him to patients like Jamie, who knows full well she’s been in the best possible hands.