Patient Stories: Carter Mae Kennedy
On October 26, a petite 4-year old girl walked through UNC hospital, dressed as Disney’s Princess Elsa. Her body vibrated with excitement as her long blue train trailed behind her, her blond wig braid bouncing on her back as she held a pumpkin bucket tightly in her hands. Carter Mae Kennedy was celebrating her most favorite holiday, Halloween, and instead of knocking on doors in her neighborhood as most children do, she was going from pharmacy to pharmacy at UNC Medical Center, her excitement growing as her little bucket filled with candy. Her 8-year-old big brother, AP, (dressed as a killer clown) and her parents Lindsey and Stephen accompanied her. It was not the usual site someone would see around the hospital, nor was it a regularly scheduled event, it was a special case for a very special little girl.
As she walked from pharmacy to pharmacy, her mother Lindsey, a pharmacist who works at the hospital, was thrilled to be able to give her daughter this gift of normalcy, just days before she was to undergo major surgery. The UNC pharmacy department had organized this unusual Halloween event just for Carter Mae and her brother, an experience they would have otherwise missed.
Carter Mae Kennedy looked like any other little girl enjoying her Halloween celebration. However, in just four more days she would be on the operating table. Back in September 2018, a rapid heartbeat, shortness of breath and a slight redness to her face were the only symptoms that hinted something was wrong inside of her little body. Melinda, the Kennedy’s nanny, let the parents know that she had witnessed the little girl having short 30-second episodes exhibiting these behaviors. After Lindsey noticed an intense flush on Carter Mae’s face one evening after coming home from work, she decided to take her to the UNC Urgent Care.
“She’s in a half day pre-k program, so I assumed it was something viral,” says Lindsey. “We were seen immediately at the Urgent Care, and the nurse was checking her vitals and trying to see what her O2, or (oxygen saturation) levels were. I could see she (the nurse) was playing with the machine a lot, changing out the machine, changing batteries, thinking that there must have been something wrong with the machine itself. She just couldn’t seem to get a correct reading. Dr. (Anne) Mounsey came in, and she listened to Carter Mae’s chest and breathing. She also tried to get O2 stats. At this point, she said ‘I’m not sure what’s going on, but I want you guys to get a chest X-ray so I’m going to send you up to the emergency room and I’ll call ahead to make sure that they know everything you need.’ ”
Lindsey and Carter Mae were seen right away at the UNC Pediatric Emergency Room. The nurse again took Carter Mae’s vital signs, and it was then that Lindsey realized there wasn’t anything wrong with the machine. The machine, designed to measure oxygen saturation, gave a clinical measurement that determines what percentage of a patient’s red blood cells are saturated with oxygen after passing through the lungs. It’s a result that reflects not only how well a patient’s lungs are working but also how effectively oxygen is being delivered to all parts of the body. In a healthy child, the oxygen saturation levels will be between 96% and 98%. Carter Mae’s 02 levels were at 85%.
“As the doctor was asking me all these questions,” says Lindsey, “suddenly a bunch of people started rushing into the room, and I began to panic. As a pharmacist here, I respond to codes so when I saw people rushing in, I knew something was wrong. I honestly was half expecting them to intubate her at any moment.”
The doctor pulled Lindsey aside and let her know that after doing an ultrasound, they could tell something was in her abdomen, they didn’t know what yet but they would figure it out. Scans were run over the next few hours as Lindsey waited by her daughter’s side in the Pediatric Intensive Care Unit, PICU. By the next morning, Lindsey and her husband Stephen were delivered a devastating diagnosis that Carter Mae had cancer. With additional testing, the doctors diagnosed her with Wilms Tumor, a rare kidney cancer found in children around the ages of 3 and 4. The American Cancer Society states that there are about 500 to 600 cases of Wilms tumor diagnosed in the US each year. Carter Mae’s cancer, however, was even rarer, a metastatic stage 4 cancer, meaning it had spread outside of her kidney. Her cancer had spread from her kidney up her inferior vena cava into her heart. The inferior vena cava, or IVC, is a large vein that carries deoxygenated blood from the lower and middle body into the heart.
“Carter Mae had a very rare case,” says Dr. Mahesh Sharma, Co-director of the NC Children’s Heart Center and Section Chief of Congenital Cardiac Surgery at UNC Medical Center. “Before she had chemotherapy, the tumor was not only going up the inferior vena cava into the right atrium of her heart; it was going through the valve called the tricuspid valve into the right ventricle. Meaning every time her heartbeat the cancer was moving from the right atrium into the right ventricle. That’s exceedingly rare; it happens less than 1% of the time.”
Fortunately, Lindsey and Stephen were also given some very good news. Dr. Stuart Gold, Chief of the Division of Pediatric Hematology-Oncology at UNC-Chapel Hill informed them that while the diagnosis was serious, it was also curable. The multidisciplinary team at UNC including Dr. Gold, Dr. Sharma and Dr. Andrea Hayes-Jordan, Surgeon-in-Chief of the NC children’s hospital and pediatric surgeon, came together to create a plan for Carter Mae to treat the disease. They started her on chemotherapy immediately to shrink the size of the tumor and scheduled her surgery for October 30th to remove it.
Before chemotherapy, the tumor on her kidney was about the size of a small watermelon. After chemotherapy the tumor shrank significantly, going from about an inch in diameter to around 3 or 4 millimeters in diameter on the IVC.
The day of the Carter Mae’s surgery was quickly approaching. “We orchestrated it out as a team, so we met ahead of time to pre-plan the operation,” explains Dr. Sharma. “We brought in all the groups that were necessary including the perfusion team, Dr. Hayes-Jordan’s team and our team to do a type of dry run before the day of the surgery. We wanted to make sure we were prepared for all scenarios.”
“I was just really floored by the fact that these three very knowledgeable physicians, Dr. Hayes-Jordan, Dr. Sharma and Dr. Gold, were able to come together and decide the best course of treatment. As a former OR pharmacist, I know that different teams can disagree on a course of action. When we went for the pre-op meetings with Dr. Hayes-Jordan and Dr. Sharma, they both exuded confidence that they’d done this before and were not worried about the surgery. That really set my mind at ease. I had zero concerns; I trusted them completely in the OR with my child.”
The original plan was to have Dr. Hayes-Jordan, the general pediatric surgeon who specializes in cancer surgery, remove the tumor from her kidney and try and pull the tumor out of her heart and off the IVC. Dr Hayes-Jordan said “some surgeons will never do a case like this in their career, but myself and Dr. Sharma have.” If there were any issues with removing it from her heart, Dr. Sharma, the pediatric cardiothoracic surgeon, would step in to perform heart surgery.
On October 30th Carter Mae was wheeled back to the operating room. Dr. Hayes-Jordan begin removing the tumor from her kidney, part of the IVC, as well as other organs. “When I got her in the operating room,” says Hayes-Jordan, “we found that the tumor was also growing into the colon and the spleen, so that part of the operation took longer than expected because I had to separate it from those organs. It was unexpected because the imaging had not shown that complication.”
Lindsey and Stephen waited anxiously in the crowded operating room. Throughout the morning the doctors kept them informed on Carter Mae’s progress. “They would call my phone from the operating room letting us know she was doing fine and everything was going smoothly,” says Stephen. “They called maybe 2 or 3 times to keep us updated on her progress. When we didn’t hear from them for a while, it was stressful not knowing what was going on, but we figured Dr. Sharma had to step in to do his part.”
When Dr. Hayes-Jordan tried to pull the tumor off of the IVC and out of the heart it could not be removed easily. Because Carter Mae’s tumor involved the vena cava and heart, Dr. Sharma had to suspend blood flow to and from the heart and vena cava. He and his team did this by cooling Carter Mae’s body temperature down to 18 degrees, essentially stopping circulation and blood flow in the body, a procedure known as a circulatory arrest. He then opened the right upper chamber of her heart (atrium) and removed the rest of the tumor from inside her heart and from within her IVC.
After 10 hours, the surgery was complete. For the pain management part of the case, the pediatric anesthesia team stepped in to help the family. They also provided them with a referral for a psychologist that specializes in children with medical illnesses. The family was grateful for this opportunity to begin working on the mental health aspect of their recovery. “Again, here was another team coming in, providing awesome care,” says Lindsey. “So that was 4 for 4, General Surgery, CT Surgery, Oncology, and Pediatric Anesthesia. Everyone was amazing.”
The support of the doctors and nurses in treating Carter Mae was a critical component of her patient care and recovery. Moreover, others around the hospital and in the community who knew the Kennedy family also showed their support. The family’s local church helped by providing a meal train as well as a housekeeper for several visits. The psychiatry residents gave Lindsey a gift card to Starbucks, but it was Lindsey’s colleagues in the Pharmacy Department that provided a whole different level of support to the family.
“My department went above and beyond anything I ever expected,” says Lindsey. “My office mate Taryn, a neurology pharmacist and Bobbi Jo, the inpatient infectious disease pharmacist got together and sent an email out to my department letting them know our situation. Carter Mae’s birthday is November 3rd, which was four days after her surgery. So I planned a little something special for her while we were still in the hospital. Taryn brought in an enormous amount of gifts; it looked like we were having four Christmases. They got stuff for Carter Mae, me, Stephen, and her brother AP. They didn’t leave anybody out. It was so generous, thoughtful and uplifting to my family and me.”
The week before Christmas the family got the good news that there was no evidence of the disease; Carter Mae was cancer free! She continues to grow stronger each day, and is slowly going back to school, a spunky 5-year-old who happened to go through open-heart surgery and who would dress up as Elsa everyday if she could.