The road to a cystic fibrosis diagnosis for Kyra

By Amy Schwanz

My daughter, Kyra, was diagnosed with cystic fibrosis (CF) in September 2004 when she was 3 months old. She was diagnosed through a sweat test done at Children’s Hospitals and Clinics of Minnesota in St. Paul. It was Kyra’s “failure to thrive” that prompted the sweat test. Since other possible causes of her inability to gain weight were ruled out, it was the natural next step/test. After Kyra’s pediatrician gave Mark and me the test results and diagnosis, he comforted us, explained the disease at a high level, and informed us that we were fortunate to live near two excellent CF Care Centers. He also immediately scheduled an appointment for Kyra at Children’s Respiratory and Critical Care Specialists (one of those two excellent care centers).

Mark, Kyra and I met Cindy Brady and Sandy Landvik at Children’s Respiratory and Critical Care Specialists (CRCCS) the morning after Kyra’s sweat test and diagnosis. That first visit, Mark and I were educated about CF, and Kyra had a physical exam. Specifically, Cindy explained the genetics behind the disease and how CF impacts different areas of the body. This helped us understand how the disease resulted in Kyra’s failure to thrive. At that first visit, we were given the option of having Kyra be an inpatient at Children’s in Minneapolis for a few days for testing and education, or for Kyra to continue as an outpatient, which meant we would come back to the clinic/hospital every few days for testing and education, spread out over a few weeks. Since we had another young daughter at home, we elected for the outpatient option. We brought Kyra to CRCCS quite frequently. At first it was every few days and then the time between visits extended as Kyra became healthier, with the visits happening weekly, and, finally, monthly. Although this happened nine years ago, I can still recall how patient, comforting, and reassuring the entire CRCCS staff was with us during those first visits. It was a very difficult time for us, but it would have been much worse without the support and care of the CRCCS staff.

Following the treatment plan prescribed by CRCCS, Kyra VERY rapidly gained weight and finally looked like a healthy baby with pink chubby cheeks. She also didn’t cry as often, slept better, and became a much happier baby. This change literally happened in days; it was amazing!  Over the course of the first few months, Kyra had several diagnostic tests done (chest X-ray, blood work, etc.) to determine her health status. Although these tests were important, more vital was for Mark and I to learn how to care for Kyra. It is not easy to care for a child with CF and it takes a lot of time to learn it all.  We learned the how and why of everything we did for Kyra – from pancreatic enzyme replacements to bronchial drainage techniques. We also worked with all of the CF care team members associated with Children’s to get the specific information about respiratory care, nutrition, etc.

Over the past nine years, our family has fallen into the routine of life with CF. Kyra has certainly had some ups and downs, as is to be expected with a chronic life-limiting illness. But, for the most part she has remained very healthy, and Mark and I are successfully managing the many challenges–medications, respiratory treatments, clinic visits, school health plans, travel considerations and medical bills. Kyra continues to have appointments four times per year at CRCCS, with one of those being her “CF annual visit” where we meet with the entire CF care team. Over our many visits to the clinic and Kyra’s one inpatient procedure (sinus surgery) in 2009, we have become very familiar with Children’s and CRCCS. I cannot say that every interaction at the hospital and clinic over the years has been perfect, but I can say that from the office staff, to surgical staff, and certainly to our great CF care team, I am frequently impressed with the level of care Kyra receives. The CF care team works closely with us to do what’s best for Kyra.

In 2004 when Kyra was born, CF newborn screening was not being done in Minnesota. On one hand, I wish that Kyra would have had the screening and been diagnosed at birth so that she would not have had such a rough start to her life. However, on the other hand, while Kyra’s diagnosis was devastating, it was also somewhat of a relief for us— to finally determine what was causing her inability to gain weight and to be able to “fix” the problem and see an improvement in her health. I think of the parents whose newborns are diagnosed, they must not have that sense of relief that we did and I think that would make the diagnosis more difficult. But, those parents also do not have to go through the first few months of their child’s life wondering why they won’t gain weight and they can get started on treatments right away, improving their outcome. I empathize with parents whose children are diagnosed with CF – at birth or later in life –because no matter when the child is diagnosed, it is a devastating diagnosis. It is a challenging road they will travel. Fortunately, with Children’s CF care team, they will, like us, have an excellent medical support system to help us along that road.

 

 

 

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