Ainsworth boy battles rare genetic disorder

Amanda and Waylon Schultz of Ainsworth became the proud parents of Payton Schultz in August 2005. Before his first birthday, the young couple noticed something was wrong with Payton. He experienced intense fevers accompanied by swollen lymph nodes and mouth blisters.

Payton?s fevers were diagnosed as an extremely rare genetic disorder known as ?HIDS,? short for Hyperimmunoglobulinemia D Syndrome. Payton has seen doctor after doctor ever since his diagnosis. He goes to the National Institutes of Health (NIH) in Bethesda, Md., for checkups four times a year. On one of his trips, he got to meet President Barack Obama.

Waylon is a native of Ainsworth, and Amanda has lived there since eighth grade. The two have been together ever since their sophomore year in high school. The couple has three children: Kaitlyn, age 10; Sarah, age 8; and Payton, age 5.

Payton was born five weeks premature with one collapsed lung and a hole in the other lung. Unfortunately for Payton and his family, his problems were just beginning. Starting at around 8 months of age, he would get terrible fevers. Sometimes the fevers lasted two weeks. They came without warning, too. Payton could go to bed in good health and wake up with full-blown fever symptoms.

?His joints would swell. He wouldn?t eat for days at a time. And he started having seizures, too,? said Amanda. ?He couldn?t move, and he would just stare at you like a zombie.?

Payton suffered from arthritis, mouth ulcers and a body temperature upwards of 107 degrees. He was taken to the University of Iowa Hospitals and Clinics (UIHC) on a monthly basis for a year after the first episode. He was there for usually a week at a time.

?The doctors made sure he was safe,? said Amanda. ?He was on the bottle but he couldn?t swallow because of his mouth ulcers. They made sure he was getting his nutrients. He was in there mostly for observation. There wasn?t much they could do for him.?

His parents even took him to the Mayo Clinic in Rochester, Minn. The doctors could not figure out what was causing Payton?s fevers.

For Amanda and Waylon, the scariest part of this ordeal was that the cause of Payton?s fevers was unknown.

?Our biggest concern was that there were no answers,? said Amanda. ?We could deal with what we knew. It was dealing with the unknown that scared us. There is nothing scarier than not having answers.?

Waylon remarked, ?The doctor comes in and tells you, ?We don?t know what we should do next.? Now what do we do? Just sit here and watch him??

?We didn?t know if he was going to live,? said Amanda. ?We didn?t know what to expect from day to day. Not to mention that his sisters were having to watch it.?

Pediatric Rheumatologist Polly Ferguson at the UIHC took an interest in Payton?s case and lobbied for him to be included in a study at the National Institutes of Health (NIH) in Bethesda, Md. At 1 ½ years of age, Payton was accepted for the study.

?Had it not been for Polly, we would not be where we are today,? said Amanda.

Payton?s condition was diagnosed as Hyperimmunoglobulinemia D Syndrome, or HIDS. Payton has a checkup at the NIH every three months, and continues to make frequent visits to the University of Iowa Hospitals and Clinics.

Payton, of course, is no fan of his daily shots. But he doesn?t mind visiting the hospital in Bethesda because of the comfortable accommodations there.

?We stay at a place called the ?Children?s Inn,? which is just like the Ronald McDonald House [in Iowa City],? said Waylon. ?They have a tree house inside and all kinds of stuff for him to do. He looks forward to those trips.?

There are essentially no standard medical treatments for HIDS because it is so rare. Payton is one of only six people in the United States who have HIDS, and one of 150 in the world with the disorder. Nearly all of the procedures to treat HIDS are experimental.

Though there are few patients who share Payton?s disorder, he is unique from the others in that the medications do not work as well on Payton as they do on the other HIDS patients.

?They want the medication to reduce his episodes to two or three per year at the most,? said Amanda.

From Payton?s point of view, the medicine is just responsible for painful side effects.

Payton has already undergone surgery in his young life, and he is about to undergo it again. He had his tonsils removed so he could swallow more easily.

?He can eat through his episodes now, which he wasn?t able to do before,? said Amanda.

His next surgery is on his heart, and it is scheduled to take place tomorrow in Bethesda.

?His heart rate goes to 200 beats per minute at times, and he gets sick to his stomach,? said Amanda. ?He?ll reach to his chest and say, ?My heart hurts.? You can see his heart beating through his shirt.?

The Schultzes have a high school friend who lives in the D.C. area. Her boyfriend was a police officer there. On one of the family trips to D.C., Payton met the firefighters, rode in a helicopter and played with the dogs in the K-9 unit.

?He hung out with the SWAT Team, too,? said Waylon.

The firefighters gave Payton a real firefighter helmet, which Payton wears proudly around the house.

?The chief of the Fairfax County Fire Department got down and took his pin off of his own jacket and he said to Payton, ?On behalf of the Fairfax County Fire Department, I?m going to present this pin to you, and from now on, you take care of your family and we?ll take care of you.? We all had goose bumps,? said Amanda.

Payton has a care page on the Internet that contains information about him and his condition. Many of the D.C.-area law enforcement officers have gotten to know Payton well and now leave messages on his page.

Through their connections with law enforcement, Payton even got to meet President Barack Obama. Obama was at someone?s personal residence for a private meeting, and Payton was on the guest list. The president turned to Payton, gave him a high-five and asked, ?What?s up, buddy??