Hydronephrosis: Maddie's Story

Maddie was a busy high school junior who took great joy in helping others. When an unrelated medical issue led to a diagnosis of chronic kidney failure, she was dismayed to find that she was the one who needed help.

The diagnosis was severe hydronephrosis, a condition in which the area of the kidney where urine is collected is enlarged. A nephrectomy was scheduled at Children’s Hospital of Philadelphia (CHOP), and just over two weeks after having her kidney removed, Maddie was able to start her job as a camp counselor for kids with Down syndrome.

“When I was put in that setting, I was forced to not worry about myself as much,” Maddie, now 20, remembers of the weeklong camp. “It kind of makes your problems seem small, even though what I had was pretty big.”

An unexpected hydronephrosis diagnosis

At the beginning of her junior year of high school, Maddie began getting headaches. As they became progressively worse, Maddie’s mother, Dawn took her daughter to various medical specialists. Along the way, a pelvic ultrasound ordered to determine the cause of additional medical issues showed an enlarged kidney compressing an ovary. Their physician suggested they get Maddie to a urologist, and the family sought expert care at the Division of Urology at CHOP.

Maddie saw pediatric urologist Christopher J. Long, MD, who ordered a renal ultrasound and a magnetic resonance urography (MRU) exam to get a detailed picture of her kidneys, ureters (tubes that drain urine into the bladder) and bladder. The exams revealed that Maddie had hydronephrosis caused by an obstruction, most likely where the kidney and the ureter meet (the ureteropelvic junction, or UPJ).

Dr. Long explained that the condition was likely present at birth and went undetected for so long because Maddie didn’t suffer any common symptoms like flank pain or urinary tract infections. The massive enlargement (dilation) of the part of Maddie’s kidney that stores urine had essentially made that kidney non-functional, and she was also diagnosed with chronic kidney disease (hydroureteronephrosis).

When life brought me and my family to the Urology Center at CHOP, we finally were given answers.

Developing a treatment plan

Treatment of hydronephrosis depends on its severity. Given the severe nature of the dilation, Dr. Long brought Maddie’s case before a panel of eight CHOP urologists and three radiologists.

The MRU image showed that her right kidney was so enlarged that it stretched from beneath Maddie’s right arm to her pelvis. It was crowding out her reproductive organs. After examining the images from the MRU, the panel was unanimous: the kidney had to be removed.

Dr. Long explained to the family that the surgery to remove the kidney would be done laparoscopically (a minimally invasive surgery technique), but the recovery was likely to be difficult given the degree of dilation and the length of time it had gone untreated. Maddie’s main concern was that she wouldn’t be able to meet her commitment to be a counselor at the PALS Program summer camp. Dr. Long prepared Maddie for the possibility that she would not be well enough in time.

“He told her that it would be a stretch,” remembers Dawn.

Recovery, then right back to helping others

The surgery lasted seven hours. Despite the kidney being the largest non-tumorous kidney ever removed at CHOP, Dr. Long was able to remove it in one piece, says Dawn.

Maddie recovered at the hospital for five days following the operation. Her bright spirit continued to shine during her recovery as she was cracking jokes and finding reasons to laugh while doing laps around the hospital floor.

One of the things that stands out most from her stay was how fabulous the nurses were. “She referred to it as ‘Hotel CHOP,’” remembers Dawn.

"People always ask me if I was scared for the surgery, but I was not," says Maddie, crediting the trust and respect she and her family felt with Dr. Long and the entire team. "I never felt more at ease with a decision in my life."

When Maddie got home, she took advantage of the downtime to write her college application essays and focus on getting better. Just 16 days after her surgery, she was well enough to report to her job as a camp counselor.

“Maddie is resilient, but without CHOP and Dr. Long specifically, we wouldn’t be where we are today,” says Dawn. “This I believe wholeheartedly. We are so very grateful and thankful.”

Not slowing down

Maddie continues to follow up with the Division of Urology and the Division of Nephrology to monitor her chronic kidney disease, as well as the Division of Neurology to manage what turned out to be chronic migraines. She returns for appointments both at CHOP’s Main Campus in Philadelphia, and closer to home at the Specialty Care & Surgery Center, Brandywine Valley.

While Maddie didn’t have her surgery at the Brandywine Valley location, the practice offers ambulatory and general surgery services, with a full team of CHOP pediatric surgeons, pediatric anesthesiologists, and specially trained nurses.

Despite these obstacles, her health has hardly held her back. Maddie is president of her school’s Peer Counselors Club, in which students mentor disadvantaged children in the school district; as well as president of Peer Pals, a group that arranges activities with club members and special needs students. She also co-runs a special needs soccer clinic and is the manager of her swim team and a swim instructor.

In addition to fulfilling her commitment to be a summer camp counselor for kids with Down syndrome, Maddie had another post-surgery goal: to walk 60 miles in the three-day Susan G. Komen Breast Cancer Walk. In November 2017 — six months after her nephrectomy — Maddie traveled to San Diego and completed the walk with her mother, grandmother and a family friend.

"I wouldn’t have been able to walk those 60 miles with such grace and intent if it weren’t for Dr. Long and CHOP as a whole," says Maddie.

The next chapter

In Dec. 2017, Maddie got the news that she had been accepted to St. Joseph’s University (SJU) — her dream school! She's seized the opportunity to say the least: Today, she is a sophomore pursuing a degree in Political Science with a minor in Autism Studies. She is co-president of the Women's Leadership Initiative, works in SJU's Kinney Center for Autism Research and Support, and is working on an independent study looking at peer institution models to implement a food pantry on SJU's campus.

Another current project she’s working on is a year-long “Focus on Women” series at SJU for which she's serving as chair of the Film Series Subcommittee, and is responsible for choosing films each month to showcase prominent women who were most likely written out of the history books.

“This kid has such incredible gifts to share,” says Dawn. “She has big plans and doesn’t have time to be ill. We call it a little bump in the road.”

Thanks to the CHOP team, I am able to live my everyday life with intention and intensity for other people. My experience at the Children’s Hospital of Philadelphia couldn’t have been better.

Updated April 2020