Spring 2007

Kendra's Story
Determined to overcome adversity

This is the first in a series of articles The Ambassador will share in 2007 about Kendra Borgerson’s efforts to conquer Prader-Willi Syndrome, a rare genetic disease that causes both medical and behavior problems. With the help of ACCC staff and a diligent family, Kendra has made great strides, but it hasn’t always been easy.

When Kendra Borgerson was born 18 years ago, she couldn’t eat. By the time she was 4 years old she couldn’t stop eating.

It wasn’t until she was 8 that her parents found out why.

Kendra spent the first six weeks of her life in an intensive care unit. She had a difficult time swallowing, making drinking from a bottle nearly impossible. She had very weak muscle tone.

As an infant, Kendra’s parents were told she was a  "floppy baby".

Doctors, said Kendra’s mom Peggy Sorge, couldn’t give any real concrete answers as to why, telling her only that Kendra was delayed and would catch up. "They called her a floppy baby, " Peggy said.

In the first few weeks, just opening her eyes was a major accomplishment for Kendra.

Once home, she still faced many challenges. She had to be positioned very carefully when being fed or her breathing would be cut off. Doctors recommended tube-feeding (through a tube placed directly to her stomach) to ensure Kendra received adequate nutrition, but her parents said no.

Instead, Peggy said, they made bottle holes bigger and fed her every three hours with a double-strength formula mixture. At 12 months, Kendra weighed only 12 pounds.

And her weak muscle tone caused many physical delays. She was 1 before she was able to sit up on her own and over 2-yearsold when she took her first steps. By then, Peggy was expecting her third child. "I just remember hoping and praying she would walk by the time I had the baby, " she said.

By the time Kendra was 4, things started changing. She began to eat uncontrollably and exhibit other behaviors that were not normal—excessive tantrums topping the list.

Finally, an answer

At age 8, one doctor finally did a genetic test that diagnosed Kendra with Prader-Willi Syndrome (PWS).

It is described on the Prader-Willi Syndrome Association website as "a complex genetic disorder that typically causes low muscle tone, short stature, incomplete sexual development, cognitive disabilities, problem behaviors, and a chronic feeling of hunger that can lead to excessive eating and life threatening obesity.

"In addition to their involuntary focus on food, " says the website, "people with PWS tend to have obsessive/compulsive behaviors that are not related to food, such as repetitive thoughts and verbalizations, collecting and hoarding possessions, picking at skin irritations, and a strong need for routine and predictability.

"Frustration or changes in plans can easily set off a loss of emotional control in someone with PWS, ranging from tears to temper tantrums to physical aggression. The essential strategies for minimizing difficult behaviors in PWS are careful structuring of the person’s environment and consistent use of positive behavior management and supports. "

With a diagnosis at hand, her family finally had an answer and saw that Kendra was a textbook example of a person with PWS. Everything they’d experienced with her since birth fit into that mold.

But having an answer didn’t make dealing with the disorder any easier.

By the time Kendra was 4, she was unable to control her eating and began having excessive tantrums, common characteristics with Prader-Willi Syndrome.

An institutional home

By the time Kendra was 7, Peggy had to begin taking drastic measures to keep food out of her reach. Cupboards were locked, a locked chain was placed around the refrigerator and the garbage was locked. Peggy kept the key with her at all times.

When Kendra began sneaking into the bedroom at night and taking it off the nightstand, Peggy began sleeping with the key.

One early morning, Peggy found Kendra outside in the cold going through the garbage there. They put a monitor on her bedroom door, but she soon figured out how to disarm it.

"There always had to be a constant watch on her, " said Peggy, in order to keep her away from food, which, she said, was on Kendra’s mind 24 hours a day. "I always had to be aware of where Kendra was and what she was doing. Even at night, I would sleep for awhile but then wake up to check on her to make sure she wasn’t in the kitchen seeking out food or going outside and wandering. "

As Kendra began to grow, her behavior began getting erratic, a common occurrence in people with PWS. "She was having tantrums many times during the day, " Peggy said. The stress of caring for Kendra, as well as meeting the needs of her two boys, was beginning to take its toll.

The breaking point for Peggy, though, was seeing Kendra’s reaction when her younger brother, Lucas, would go to play with friends.

"She didn’t have friends, " said Peggy.

She was invited to others’ homes a couple of times to play. "But with her syndrome, she could only stay for a while and I think it was more than they could handle. She wasn’t asked over a second time, " Peggy said. "I’m not sure who hurt more, her or me. I could tell that Kendra wanted someone to play with and have friends at school. "

With PWS, Kendra does have a cognitive impairment and was receiving special education services in public school. While Kendra can talk, she was difficult to understand for those who didn’t know her well.

"She had really good teachers but they didn’t, and I didn’t, understand how to teach her. Her behaviors got in the way of her learning, " said Peggy. Kendra would go through lockers and teachers’ desks looking for food at school and tantrums were an issue there, too.

But the most difficult thing for Peggy was hearing Lucas, in tears, talk about the way other kids were treating Kendra. "It was to the point I didn’t want her there (at school) because I was worried about her and her feelings, " said Peggy. "I was really scared about her going to middle school, starting with new teachers and kids and wondering how they would treat her. "

It was around this time Peggy, now a single parent trying to hold down a full-time job working from home, began looking for help beyond the occasional respite care she had coming into her home.

"The day I took Kendra to ACCC to live was mixed with different feelings, " she said. "I had given up trying to care for her and felt I failed her. How was Kendra going to feel about my leaving her? "

In the summer issue of The Ambassador, find out how Kendra felt and how she began to change with the help of a highly-structured living and school setting.