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Francis Barrette died in 1990 of throat cancer, some 21 years before a devastating diagnosis came to light of an illness that affects 11 members of his extended family ranging in age from 3 to 52 years old.

So far, that includes three of his children, as well as some of his grandchildren, great-grandchildren and great-great grandchildren.

The disease is called neurofibromatosis type 2, or NF2, a genetic disorder known for uncontrolled growth of non-cancerous tumors in a person's nervous system. It affects their hearing, eyesight and balance. Depending upon the type of tumors, it can impact one's lifespan.

Members of the Barrette family have discovered all of this in just the past three years.

How it began 

Jill Pearson of Clinton struggled for years with her hearing. The sister of Jamie Dornbush of Clinton, she took a hearing test, which indicated the need for a magnetic resonance imaging, or MRI, exam. That test detected a tumor inside the skull, near one ear.

"I was with her at the MRI test results. That was difficult," Dornbush said.

Pearson died of a heart attack at the age of 57. By the time the NF2 tumor was discovered, it was too big to remove. Dornbush accompanied her to every appointment at University Hospitals and Clinics in Iowa City.

"I didn't want her to be alone out there," she said.

Dornbush, 52, works for a Clinton physician and was next to be diagnosed. It was late 2010 when a tumor on her face was removed and tested. It was positive for NF2.

Dornbush had surgery that affected her balance. She uses a cane and a walker now, and her quality of life has been affected beyond that. She has a 30 percent loss of hearing and is seeking supplemental security income, or SSI, benefits, but has been denied twice. The case is being appealed to a judge.

Older members of a family such as the Barrettes may be diagnosed initially with neurofibromatosis when an individual suffers a hearing loss. After genetic experts know what to look for, the rest of the family members can be tested, beginning with MRI exams.

A debilitating disorder

The family's care also involves the genetics department in Iowa City and Dr. Pamela Trapane, a medical geneticist.

The NF2 disorder can be debilitating. "It can be really mild or really horrible," Trapane said, noting that the hearing loss is a particularly difficult adjustment for someone who has always had hearing.

"With NF2, the tumors tend not to be malignancies. But the amount and pain and disability can be devastating. I'm amazed how people cope," the doctor added.

Dornbush's daughter, Alicia Rogis, had a tumor pushing on the spinal cord, stretching from her neck to the middle of her back. A nurse in the surgical intensive care unit, or ICU, at Trinity Rock Island, Rogis had that tumor removed in her first major surgery after being diagnosed.

Dr. Bruce Gantz, a neurotologist at Iowa, tends to several members of the Barrette family. The tumors that develop are connected to a patient's nerves, so the extent of disability depends on how much those nerves are impacted.

The nerves come out of the brain stem and can involve swallowing, the voice box or be on a facial nerve that allows a person to smile, he explained.

Genetic testing

The Dornbush case sent many members of the Francis Barrette family to be examined. However, not everyone chose to undergo the genetic test, and Trapane said that is not unusual.

NF2 is often an adult-onset condition, so it is found when a person is in their 20s or 30s. "Often they do want to get testing when they start to have children," she said. Some, however, are more comfortable not knowing.

Rogis, the nurse, was secretive about her NF2 test. She contacted Trapane's office in Iowa City without telling her family and went by herself to be tested.

"I told my mom when I found out I was positive," she said. Rogis, who is single, has decided not to have children as a result of the genetic tests.

She is recovering from a surgical procedure. The surgeon took off one tumor and half of her balance nerves, but left one alone so the young woman's face would not be paralyzed. She walks every day for exercise, hoping to return soon to her career.

"I love being an ICU nurse," she said. "I want to be a nurse as long as I can. I want to enjoy it as long as I can."

Holiday spirit? 

The holidays are an especially difficult time for the Barrette family. Dornbush, for example, said that while she likes to look at lights and decorations at other locations, she does not celebrate the season at home.

"I don't put up a tree," she said.

Wendy Buikema is a daughter of the late Jill Pearson, who was the first member of the Barrette family to be diagnosed with NF2. Buikema has also tested positive for the disorder, as has her 21-year-old son.

"It's a very sad and depressing time of year," she said.

But the family remains close, and the diagnosis has not affected their caring for one another and the degree of friendship they share. Dornbush, for example, accompanies Buikema, Buikema's son and Rogis to all of their appointments in Iowa City. Some member or members of the extended family travel to Iowa City almost every week for one health concern or another.

Rogis, for example, still has temporary paralysis on the left side on her face that stems from the recent surgeries. She cannot eat or drink on that side of her face, and when she goes to bed at night, she must tape down her left eyelid.

But the 29-year-old has found ways to cope. She loves her job and thinks about how she wants to return to it. 

"I just try and stay positive. Every single night, I run through everything good in my life, That helps," she said.

When the Barrettes visit Iowa City, they see children, including babies, who are undergoing cancer treatment. "There are lots of medical situations harder than what we have," Rogis said. "I do remind myself: Things could be so much worse."

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