Cystic fibrosis complicates toddler’s life

Kane and Janelle Faber are graduates of Mohawk High School who now live and work in Findlay. The couple’s daughter, Sophia Grace, is only 20 months old, but she has been through surgery, blood transfusions, lengthy hospital stays and numerous doses of medicine to fight cystic fibrosis.

Saturday, Moreno’s Restaurant in Carey has agreed to be the venue for a cystic fibrosis fundraiser in honor of Sophia.

The toddler’s smiling face belies the struggle that began even before birth. An ultrasound showed excess fluid around Sophia’s abdomen. The obstetrician sent Janelle to Riverside Hospital in Columbus for more tests that revealed she is a carrier of the genetic factors for cystic fibrosis. When Kane was tested, he also was determined to be a carrier of the gene mutation. That boosted the chances for the baby to suffer from the disease.

Janelle was sent to Nationwide Children’s Hospital in Columbus.

“They did an amnio(centesis). They withdrew some fluid from Janelle’s stomach and tested it. It came back that (Sophia) did have cystic fibrosis. Once the diagnosis was figured out, they had to figure out why the fluid was there,” Kane said.

A special MRI showed a blockage in Sophia’s bowel. A team of physicians at Nationwide made a plan to correct her condition immediately after her birth. Janelle’s body also became swollen with excess fluid, which had to be drained.

“Once we started going down to Riverside, we were going down every Wednesday. … 14 weeks in a row, before we ended up having her,” Kane said.

At Sophia’s 36-week check-up, the doctors were concerned about the fluid in mother and daughter. They advised Janelle to deliver the baby four weeks early so Sophia’s bowel could be repaired. She was born July 12, 2012, weighing 6 pounds, 13 ounces.

Soon after delivery at Riverside, Sophia was transferred to Nationwide.

“They sent over a special ambulance, a big vehicle, and they hooked her up and everything. Within three hours of having her, we were on our way to Children’s. Of course, Janelle had to stay at Riverside. I went over, and I was up for probably three days straight,” Kane said.

Attempts to flush the obstruction were unsuccessful. Sophia’s tiny bowel had doubled back on itself and cut off circulation to part of the organ. The surgeon removed 10 ounces of fluid, severed the deadened segment of the bowel and rejoined the healthy parts.

After three hours in surgery, Sophia spent 34 days in the NICU at Nationwide. When an e. coli infection broke out, Sophia had to be put on a ventilator. Kane said hearing the cries of the other children (and parents) in the unit added to their own distress.

“We stayed at Ronald McDonald House, and I took time off work (at a Findlay auto dealership). Then, I came back to work and every night I drove down,” Kane said. “We were raising our child in a hospital the first month and a half. Nothing was normal that we went through. She was constantly hooked to all kinds of tubes. If we wanted to hold her, we had to gather everything to do that. It was a very hard time in our lives.”

The cost of Sophia’s stay was more than a half-million dollars, but insurance covered most of the bill. Certain things, such as the respiratory syncytial virus shot, have not been approved. Special food is not covered nor are travel expenses.

“We go about once a month to Columbus at Nationwide Children’s to get check-ups,” Kane said.

After Sophia had been home for about a month, she contracted a pseudomonas infection in her lungs. The infection recurred three more times, requiring a round of antibiotics each time. Kane said there always is a risk of his daughter developing an immunity to the antibiotics. One of her nebulizer medications costs $12,000 per month, in addition to nearly 20 pills a day and inhalers.

“She gets breathing treatments three or four times a day for 30-45 minutes each,” Kane said. “Now, she has a vest that’s hooked to a machine that shoots air. The vest vibrates her (to loosen mucus), but before she was a year old, we had to do everything by hand.”

Janelle also applies essential oil on Sophia’s feet at night to promote better breathing. Even with constant care, Sophia often becomes ill from the thick mucus that forms in the respiratory system and the pancreas.The mucus remains in her body as a breeding ground for bacteria. Almost a year ago, Sophia’s lung oxygen level dropped too low. She spent about two weeks in the hospital for intravenous antibiotics.

While Sophia was hospitalized, Kane started doing research online out of “an urge to do something.” He discovered the Great Strides Foundation, which conducts benefit walks in various cities. The Fabers participated in the Columbus walk last year. With only two months to collect donations, they were able to raise $14,000.

“I’m not a doctor. I can’t do the research, but I sure as hell can raise money for the people who can do it,” Kane said.

The Fabers are hopeful the Cystic Fibrosis Foundation will enable new treatments to be discovered. Kane said medications are being developed to help the mutated genes function in a more normal fashion. In the 1980s, children with the disease were not expected to go to school. The lifespan of CF patients is longer now, but still short at 40. Some can only survive with a double-lung transplant – if one becomes available.

Sophia is doing relatively well – walking, growing and exploring her world. In addition to words, she can speak in the sign language Janelle has taught her. Kane said they must enrich Sophia’s diet with olive oil, avocado and other high-calorie additives. They read all the food labels and choose snacks that are the highest in calories.

“Before she eats, she gets enzymes to help break down the food. … the pancreas is insufficient, so she doesn’t absorb the nutrients in food like she should. Everything stays fatty going out. That will stick and bind up,” Kane said.

This week, the couple and their friends and family are gearing for the Italian buffet fundraiser 5:30-8:30 p.m. Saturday at Moreno’s. Kane said his mother is related to the restaurant owner. The owner agreed to prepare the dinner at a reduced cost and to donate 50 percent of alcohol sales to cystic fibrosis. Local businesses have contributed raffle and auction items as well. The dinner is $10 for adults and $5 for those age 10 and younger.

May 4, the Fabers will donate proceeds from the event to the Cystic Fibrosis Foundation at its annual walk in Columbus. The Faber team’s goal for this year is $10,000. So far, they have surpassed $7,600.

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