In battling a rare blood disease, Benjamin Wayne has spent many a childhood day checking into hospitals for blood transfusions.

About 25 of them, to be exact.

But nowadays, following a spleen surgery and treatment, the 11-year-old is more focused on Boy Scouts, Star Wars, Legos and starting sixth grade this fall at Reed City Middle School.

“I feel like I’m on top of the world,” Benjamin said from the living room in his family’s Reed City, Michigan, home.

Details, details

Precocious and talkative, Benjamin can tell you every detail of his medical journey—all the dates, places and times.

For instance: He remembers the exact moment Daniel Watkins, MD, a pediatric surgeon with Pediatric Surgeons of West Michigan, removed his extremely enlarged spleen in an operating room at Spectrum Health Helen Devos Children’s Hospital.

About 12:30 p.m., March 11.

His spleen should have been about 8 centimeters long. But it had ballooned to 20 centimeters—the size of a football, Dr. Watkins said.

It stemmed from a rare hereditary disease called spherocytosis, which causes the body to produce abnormal red blood cells.

This put Benjamin’s spleen in overdrive as it tried to clear his blood.

It led to fatigue, jaundice, severe anemia, gallstones and an enlarged spleen. Add to that an unrelated diagnosis of nephrotic syndrome—a kidney disease requiring high-dose steroids for treatment—and it meant Benjamin felt pretty lousy.

Until that March day when Dr. Watkins removed his spleen.

“I feel the best,” Benjamin said. “He really changed my life, or otherwise right now I’d probably be in the hospital getting a transfusion.”

Dr. Watkins said Benjamin’s spleen had been one of the larger he has seen in a child his age. He removed it using a minimally invasive technique called laparoscopy.

“We knew it was going to be big based on the imaging,” Dr. Watkins said. “But it was a little bigger than I thought it would be. Still, things went very smoothly in the operating room.”

He placed a bag inside Benjamin’s abdomen, cut the spleen into pieces, placed the pieces in the bag and then removed it all through three small incisions and the belly button.

This meant less recovery time, less pain and fewer post-surgery risks of infection or hernia.

Benjamin’s condition had also caused him to develop gallstones, which meant Dr. Watkins had to remove his gallbladder, too.

He is already enjoying his new life.

“I’m happy he’s doing so well,” Dr. Watkins said.

Family history

Benjamin’s mother, Katie, who also has hereditary spherocytosis, said doctors diagnosed Benjamin with the disease at age 5, about the same time he started showing symptoms.

The young boy had recently lost his father around that time.

Benjamin’s younger brother, Brad, also has the disease but so far has not exhibited symptoms. In Katie’s case, doctors removed her spleen at age 5, as it had started to rupture.

Benjamin had his first blood transfusion in 2014. The transfusions are necessary to help replenish his hemoglobin levels when they drop to dangerous levels.

His health dipped several times throughout his childhood, often when he would catch common illnesses that were harder on his body than on a typical kid’s.

It reached a crisis level in February.

He got the flu and his hemoglobin levels dropped. His condition deteriorated quickly, leading to weakness and delusions.

“I asked a weird question to my Grandma about, ‘Was there school in the 1950s?’” Benjamin said. “Then I got up because I had to go to the bathroom, but I just stood right (in the living room) and didn’t move.”

“It was very bad,” Katie said. “By the time he got to Grand Rapids, his hemoglobin was a 4.”

He spent a week in the pediatric intensive care unit at Spectrum Health Helen DeVos Children’s Hospital.

Dr. Watkins and other doctors had by then already decided it had come time to remove the boy’s spleen, but the medical crisis reinforced their decision.

Once Benjamin felt better, doctors sent him home to spend the next month receiving immunizations needed to prepare him for surgery.

At some point during the ordeal, Benjamin named his spleen Gus.

He felt happy to see Gus go.

“I kind of hope he’s burnt right now,” Benjamin said.

Always vigilant

Living without a spleen comes with risks, as one of the main functions of the organ is to fight infections, Dr. Watkins said.

That’s why doctors like to wait until children are older to remove it, so they have built up more immunity. That’s also why Benjamin needed a series of immunizations before surgery to vaccinate against several infections.

Now, he and the adults who care for him are on high alert for any sign of illness, which can require intravenous antibiotics to fight.

He also takes a daily low-dose antibiotic—at least until he reaches adulthood, although perhaps for the rest of his life, Dr. Watkins said.

Benjamin manages his medications like a pro.

“He knows everything,” Katie said.

And he’s happy to put his days of illness behind him.

“I feel great,” he said. “I don’t even feel like anything happened.”