Teen with rare immune system condition makes most of long Duke Hospital stay
Aug. 03, 2013 @ 06:11 PM

Past the pink butterflies and fat alligators drawn on the pediatric floor windows is Derek Icenhour’s room. He watches the bustle of the nurses’ station from his bed, where he’s hooked up to an IV 20 hours a day.

The other four hours mean freedom to Derek, who has spent almost three months on the fifth floor of Duke University Hospital. His father, Tony, can say they’ve spent 129 days in the hospital this year without looking at a calendar.

Derek is only one of three identified people in the world who has this type of immune system disorder, and he is the oldest reported to have it at 15 years old. The other patients, based in Switzerland and Denver, are less than 2 years old. Problems with his immune system have doctors and researchers searching for an explanation.

His parents say he’s had medical issues ever since he was a baby, but when he was 4 years old, doctors told them there was a concrete reason for the frequent infections and spells of intense pneumonia.

Derek said he doesn’t remember much from those early years, besides going to the hospital in Pittsburgh a lot.

The Icenhour family moved to Wake Forest in 2006 to be closer to Duke Hospital. Derek’s mother, Kristin, said at the time there were only four hospitals in the country doing aggressive immunology research, and Duke was one of them.

Tony and Kristin swap off every 24 hours, sleeping in the hospital chair beside Derek’s bed. The other parent goes home to take care of Kevin, their youngest. Their oldest, Shannon, is a student at UNC.

They’re a Hokie family, since Tony and Kristin first met and fell in love at Virginia Tech. Hanging above Derek’s hospital bed is a VT backpack. A whiteboard on his door reads, in marker, “Reserved for Hokies!”

Derek has watched the young patients of the fifth floor come and go, and he’s come to recognize the familiar faces. These kids are going through chemotherapy or transplants, and Derek said he does what he can to help.

“I just try to make the best out of it,” Derek said. “I like making other kids happy in the hospital. I’ll give them a snack or a little toy…It made me feel better to help other people, to see them smile after they go through chemo or something else they’re going through.”

His face lights up when the hospital therapy dogs come around. The golden retriever or little corgis that hop up on his hospital blankets are the next best thing to his own dog at home, Emma, a miniature schnauzer that can’t visit his room. Sometimes the family will bring Derek outside to visit his dog in the hospital yard.

“I like bringing Emma to make Derek happy,” said his little brother, 11-year-old Kevin, while playing with small silver Slinky in the hospital room.

Derek keeps a book of family dog photos by his bed and a stuffed dog by his pillows. He said when he’s having a bad day, the photos him get through. The steroids in his medication right now are causing him pain in his back and shoulders, and his legs tend to cramp up unexpectedly. His expression is tired, but he smiles at the thought of pranking the Duke nurses.

He loves to irritate them, he said. He’ll put putty and slime on his stomach, and then tell the staff that his stomach hurts. He’s had a few fake seizures in front of the nurses in training, which he said freaks them out.

“They’ve learned,” Kristin said.

“Most of them,” Derek replied.

The nurses, despite the tricks, have brought him plates of spaghetti and cookies. They’ll join in midnight Uno or Bananagrams games with the family. The Icenhours even held a Super Bowl party during one of their last hospital stays, when they projected the game on the wall and ate KFC.

They’ve tried to make the hospital room a piece of home. He has his prized Pittsburgh Steelers mug that the nurses got him. His bedside table is covered with markers and snacks like Lucky Charms (he’s eating the cereal part first so he can enjoy all the marshmallows at the end).

But Derek said there are things he tends to miss. He’s a rising sophomore this year at Wakefield High School in Raleigh, yet he hasn’t been to class since Thanksgiving. He is going through tutoring and online courses, but he said he misses seeing his friends. He plays second baseman on a baseball team, but he’s had to sit out the games.

“(I miss) really not being able to see my friends,” he said. “Or sleep in my own bed.”

Kristin said they hope that their son will get to come home at the end of August, if all goes well. In a few weeks, they will wean him off of his IV medication, a mix of steroids, electrolytes and other drugs, to see if he can take the medicines orally and at home. This is one of many medications they have tried to use to combat his symptoms, which have spiraled into kidney damage, getting his gall bladder removed, low platelets, more pneumonia and pericarditis, or inflammation of the sac surrounding his heart. His stomach cannot absorb nutrition on its own.

“He’s the most complicated case they’ve seen here,” Tony said.

His condition is still a question mark. Doctors have lumped it into the description for common variable immunodeficiency disorder, which Kristin described as a catch-all for patients who show symptoms of having low levels of proteins that fight off infections. Doctors are testing if Derek’s condition may be classified as hyper IGM syndrome instead, where a patient shows abnormal levels of proteins called antibodies or immunoglobulins.

If he has hyper IGM syndrome, it’ll be a type that hasn’t been identified yet. His parents also have been going through genetics studies to try and find the cause.

“We really don’t have that much foresight of what’s heading our way,” Kristin said.

“You have to have faith that there is an end to this somewhere,” Tony said.

“I don’t know,” Derek said about how he plans for the future. “Try to be hopeful.”

He said he’s one of the most well-known patients in the hospital now and that it’s weird being one of the only people in the world with this condition.

As a result, he has requested one thing of the hospital staff: To name the disorder or disease after him.