Taking a team approach
"It's been a whirlwind," Stephanie Cooper recalled of the last three years.
In January 2015, Stephanie's then 14-year-old daughter, Erica, began having headaches that were so severe she would be in tears from the pain. Her pediatrician suspected stress and prescribed medication for anxiety. As the frequency and intensity of Erica's headaches increased, her pediatrician also noted her blood pressure was elevated.
Because Erica's dad, Mike, had a kidney birth defect, which caused headaches and high blood pressure but was diagnosed only after protein was discovered in his urine, he requested Erica's pediatrician conduct the same test. Her pediatrician found high levels of protein and referred Erica to CHoR. Two days before Erica's appointment with Timothy Bunchman, MD, professor and chief, her blood pressure became so high and her headache so severe that her parents called the rescue squad.
"The most common cause of high blood pressure in kids is kidney disease," said Dr. Bunchman, who met Erica in March 2015. "Erica had protein in her urine, high blood pressure and was in kidney failure."
Two days later, not knowing what was causing Erica's kidney failure, Dr. Bunchman admitted Erica to CHoR where she underwent a kidney biopsy and began receiving medication in an effort to stabilize her kidney function. Dr. Bunchman determined Erica did not have the same kidney issues as her dad but based on the biopsy results, diagnosed her with membranoproliferative glomerulonephritis type 3 (MPGN), a rare chronic disease where an individual's immune system attacks the kidney's healthy cells preventing the kidneys from functioning properly.
While there is no cure for MPGN, the disease can be slowed with medication until many of the patients eventually need a kidney transplant. But unlike some diseases where early transplants can eliminate the disease, Dr. Bunchman said because there is an almost 100 percent chance of MPGN recurring in the new kidney, he will wait to recommend a transplant for Erica.
"I was relieved," Stephanie said of finally learning what was causing Erica's headaches, "but I was kind of scared. I thought 'what do I do now?'"
Erica spent four days in the hospital, and Stephanie never left her side. Erica said she appreciated being able to keep up with her schoolwork while at CHoR and described visits from therapy dogs as comforting. From the beginning, Erica said the team would ask how she was feeling and involve her in her care.
"They explained every detail and situation to me," Erica said. "They told me why they did the things they did and asked me questions to determine how to help me best."
Stephanie said she appreciated Dr. Bunchman's approach to working with Erica. "He was always straight up with us. He didn't sugarcoat things. And if he didn't know the answer, he said he didn't know but would find the answer."
With prescriptions for 16 daily medications, Erica returned to her Lousia County home, but within a week, Stephanie said, "Erica started going downhill. Her face began to swell, the medications her doctors prescribed made her sick, and her weight dropped to 88 pounds."
A Team Approach
Erica began making weekly visits to CHoR, a three-hour round trip, for blood work and appointments with nephrology and gastrointestinal specialists. Erica also met with the nephrology team's nurse educators, social worker and dietician, roles Dr. Bunchman added to the team when he arrived at CHoR in 2011.
"Teenagers are an interesting population," Dr. Bunchman said. "We see kids all the time with unusual diseases. Their classmates can embrace or ostracize them, which is why having a social worker on our team is so important. [Our social worker] recently received a grant for a teen support group, which provides emotional support for the kids. They realize they aren't the only ones dealing with their disease."
Stephanie said the nephrology team's two nurse educators also were incredible support to her family. "They were there for me for every biopsy Erica had and the ER visit when we thought her kidneys were failing. They take care of Erica like she's their kid."
Dr. Bunchman, a father of five, said he believes in the importance of being honest with his patients and loving them like his own. He shares his email address and phone number with families and encourages them to reach out to him with questions or concerns.
"Having kids engaged as part of the decision makes the solution easier," he said.
Three years after her diagnosis, Erica's medications have been reduced to six pills a day, and her visits to CHoR have transitioned to every four to six weeks. While she will need medication for the rest of her life, Dr. Bunchman said her disease shouldn't slow her down or impact her future. He does not expect her to need a kidney transplant for 10-12 years, and as Erica gets older, the CHoR team will partner with VCU Health's adult nephrology specialists to transition Erica to their care.
Erica, a high school junior, is already proving her disease won't slow her down. She began volunteering with the Louisa County rescue squad this spring, enjoys taking photos of old buildings, and hopes to go into the health care field after high school. After one of the fourth graders she tutors told her about kickboxing, she decided to give the sport a try – "to show people after everything I've been through, I do not limit myself."
When asked how the specialists at CHoR have helped her and her family, Erica shared tangible examples like discount medication coupons and places for her family to stay while she was in the hospital. She also shared some immeasurable ones. "They have put their time and effort into helping me keep my kidneys as long as possible. They have been my second family and home."