After weeks of rain, high river levels and unprecedented weather patterns this fall, 18-year-old Alec Greenberg was glad to be back on the water. A member of River City Crew, Alec welcomed the sunshine and seasonable temperatures as he prepared for his team’s Friday afternoon practice on the James River.

As a high school freshman, Alec joined River City Crew’s competitive-level rowing program after his plans to play soccer for his school’s junior varsity team didn’t work out. Diagnosed soon after birth with hereditary spherocytosis, a genetic condition that affects the red blood cells, Alec often lacked the physical stamina of other boys his age. As a coxswain for his crew team, Alec relied on mental stamina to control the boat’s steering and navigation through the instructions and motivation he provided to his 4-member crew. His practice paid off in June 2017 when his team competed at its first national crew competition, one day before he had his gallbladder and spleen removed at Children’s Hospital of Richmond at VCU (CHoR).

Early Diagnosis and Treatment

Alec was born three and a half weeks early so his mom, Natalie, wasn’t surprised when his skin and eyes turned the yellow color consistent with jaundice, a common condition in newborns particularly premature ones. Caused by a buildup of bilirubin, a yellow pigment produced during the normal breakdown of red blood cells, jaundice typically resolves itself within a couple weeks. But because jaundice can also be caused by spherocytosis, and since Natalie, her mom and other family members have the condition, she and her husband, Hank, had Alec tested.

One in 5,000 individuals has hereditary spherocytosis, which can cause anemia, jaundice, an enlarged spleen and in some cases, gallstones, short stature, and delayed development. While most forms are not life-threatening, individuals with the severe form of hereditary spherocytosis require frequent blood transfusions to replenish their red blood cells, which are responsible for transporting oxygen throughout the body.

After spending an additional night in the hospital after he was born to regulate his jaundice, Alec’s red blood cell count dropped so significantly during his first day at home that he became limp and experienced shallow breathing. First-time parents Natalie and Hank brought Alec to the emergency room at CHoR where he was admitted to the inpatient floor. Alec was seen by physicians from CHoR’s Division of Hematology and Oncology, which treats children with cancer and blood disorders. Over the next six months, Alec underwent regular red blood cell count checks and blood transfusions before transitioning to annual visits.

Alec’s condition caused him to grow slowly, placing him on the lower end of the height charts, and tire easily, symptoms that were frustrating but not life-threatening. His family also watched for more concerning symptoms like high fevers and jaundice.

“Before surgery, when Alec got sick, he would take longer to recover,” Natalie said. “Between the age of 7 and his early teens, every few months Alec would develop a 3-day fever, which typically happened when he would get run down.”

Happy New Year

When Natalie was diagnosed with hereditary spherocytosis as a child, the treatment protocol was to remove a patient’s spleen, which breaks down red blood cells, around age five. This early removal was done to prevent red blood cells from getting trapped and destroyed in the spleen, causing the organ to become enlarged. However, since the spleen filters blood and helps prevent infection, early removal can increase a patient’s risk of infection.

“Some children don’t need to have their spleen removed,” said Gita V. Massey, MD, associate professor, hematology and oncology, CHoR, who began treating Alec as a newborn. “Now we observe patients until they display other symptoms.”

For Alec, those symptoms appeared on New Year’s Day 2017.

“My sister and I were walking to lunch, and she said I looked yellow,” Alec recalled. “I remembered that I hadn’t been sleeping well and had had a stomachache for three days.”

For Natalie, who was three time zones away visiting a friend, Alec’s symptoms were “concerning because I was worried about his spleen rupturing.”

Blood tests and an ultrasound revealed his symptoms were the result of gallstones, and Dr. Massey referred Alec to David A. Lanning, MD, PhD, professor of surgery and pediatrics, co-surgeon-in-chief, CHoR.

“When I met Alec, I’d never seen someone so jaundiced,” said Dr. Lanning. “His spleen was enlarged, he had gallstones and was uncomfortable.”

Although Dr. Lanning said Alec knew he would have to have his spleen removed at some point, Dr. Lanning suggested that because Alec’s symptoms were not life-threatening, he delay surgery to give his body time to make new red blood cells, a process that typically occurs every 120 days, and see how his body responded.

“I appreciated Dr. Lanning’s perspective to give Alec’s body time to adjust on its own,” Natalie said. “He also used the time to run more tests and make sure we weren’t missing anything.”

Natalie recalled how Drs. Lanning and Massey partnered to coordinate Alec’s care and how Alec and his parents were integral members of Alec’s health care team, weighing the pros and cons of surgery based on all the available medical information.

“We ultimately decided to do the surgery before Alec went to college,” said Natalie, “rather than risk him having another flare up when he was away from home.”

The Long Wait

On June 13, 2017, Natalie and Hank waited while Dr. Lanning removed Alec’s gallbladder and spleen during a nearly four-hour procedure, which was done laparoscopically to reduce Alec’s recovery time. He spent two nights at CHoR and within a week returned to crew practice. Two months later, Alec was water skiing and tubing. Six months post-surgery, his blood counts were in the normal range, and the whites of his eyes, which were yellow from jaundice for nearly four months before surgery, remained clear.

“I’m absolutely glad I had the surgery,” said Alec, who will take penicillin daily until he finishes college because of close living quarters and get annual flu and pneumonia vaccines to reduce his risk of infection. “I feel more energized and more awake.”

He’s also grown almost three inches and gained 30 pounds, nearly sizing him out of his coxswain role. (Most male coxswains weigh less than 120 pounds.) Until then, he’ll continue his senior season with River City Crew, practicing five days a week and competing in another 8-10 regattas before he leaves for college.

“We are so appreciative for the team of doctors that supported our family through this journey,” Natalie said. “The personal care, medical expertise, and collaborative discussions that Dr. Massey and Dr. Lanning provided helped us feel confident about every decision we were making for our son’s future.”