By the time a child is four, they know how to sing the alphabet, count to ten and recite their favorite nursery rhymes or songs. Most five-year-olds know their address and phone number and their parents’ names. Many five-year-olds can write their names with backward and disproportionately sized letters. Very few four-year-olds know what a kidney is, even fewer know about kidney disease. Fewer still have ever heard of FSGS: Focal Segmental Glomerulosclerosis. No four-year-old should ever have to schedule time out of their busy play days to take medicines and to know the problems that will occur if they miss a dose.
Isabelle Warrington, lovingly referred to as Izzy by those close to this precocious, loving and intelligent little girl, knows too well about Cytoxan, Cellcept, Tacrolimus and Rituximab. She can’t correctly pronounce the names yet, but she has had to deal with rigid dosage schedules, side effects and long-term implications. Izzy’s friends are warned that junk food will spoil their dinners, but she knows that the same foods have more serious consequences for her. Izzy needs to know about sodium contents and is just as likely to have conversations about her meds as Dora the Explorer’s latest adventure. She knows more than any four-year-old should ever have to know. She has been through more than any five-year-old should ever go through.
Izzy is a typical five year-old in many regards: She loves babies, both dolls and the real-life kind; she plays house and dreams of being a mommy just like her mom, Jamie. Izzy loves princesses, especially Snow White, and butterflies like those that adorn her bedroom. She likes to sing and dance; she enjoys shopping and baking; she helps her mom with chores; and she likes to take pictures and make crafts. Izzy loves chocolate and is especially fond of M&M’s, just like her Mommy. Izzy has a younger brother, Braxton, who pesters her, but she loves him anyway. Her favorite color is yellow, the perfect choice for “Team Izzy” shirts worn by all those who walked in her honor at the Kearney Kidney Walk to benefit the Nebraska Kidney Association.
Izzy’s physical problems began at birth. She was born tongue tied and was prone to illness, mostly respiratory infections and viruses. In January 2009, tests during a clinic visit showed protein in her urine, but the doctor was not too concerned at the time. Just a few months later, Izzy was hospitalized due to a viral infection. Additional tests indicated her urine still contained protein, and after more tests, she was diagnosed with Nephrotic Syndrome and began a 12-week prednisone therapy. When this treatment did not work, Izzy was referred to a Pediatric Nephrologist in Omaha, who performed a kidney biopsy, resulting in a new diagnosis: “Minimal Change Disease,” the disease that most closely matched some, but not all of Izzy’s symptoms. Several different drugs were prescribed over the course of the next few months. One was known to cause birth defects, preventing Izzy’s pregnant mom from handling it. Four others were tried, all with the hope they would suppress her immune system causing her body to "snap out of it," sending the disease into remission, but Izzy’s body did not respond to any of them. The family tried different diets, including gluten-free foods, which had been credited with helping other patients.
Meanwhile, Izzy’s protein levels were all over the chart. Constant lab work monitored every change, and each drug offered new challenges for the family whose lives rotated around drug schedules, strict dosage instructions and dealing with the side effects. In 2010, Izzy’s circle of medical professionals widened to include a Pediatric Pulmonologist due to frequent respiratory problems, a Pediatric Gastroenterologist to deal with severe stomach pains Izzy encountered daily and an Ohio colleague of the Pediatric Nephrologist who acted as a consultant during conference calls. Izzy also traveled to Houston, Texas, to see a Pediatric Nephrologist, who was considered the best in the country, in order to get a second opinion concerning a drug with known risky side effects that her current doctor wanted to try, which led to a third opinion in Denver, Colorado; genetic testing with no clear determination; and another kidney biopsy. The results, though still not definitive, seemed to move further from the initial diagnosis of FSGS, and the doctors and family now found themselves looking in a completely different direction and the possibility of beginning the process anew.
More lab procedures are pending; more trips to specialists in and out of Nebraska are scheduled; and Izzy faces more uncertainty as her symptoms and test results continue to offer mixed messages with no clear answers, just a temporary general label of "Nephrotic Syndrome." The doctors suggested an expensive commercial-grade blood pressure monitor, for her tiny arm, in order to try one more medication that her doctor is hoping will lower the amount of protein she is losing in her urine. Unable to afford the machine, the Nebraska Kidney Association was able to help using emergency patient assistance funds along with securing private donations from concerned people who heard Izzy’s story. So Izzy waits, her family worries and the doctors work to find answers.
One in six Nebraskans either has or is at risk for developing kidney disease. Kidney disease does not discriminate. People of all races, all religions, all sexes and all ages deal with kidney disease. Those with hypertension and diabetes and/or a family of history of kidney disease are at a higher risk and should be screened for the disease.
The Nebraska Kidney Association (NKA) hosts free screenings throughout Nebraska. The NKA had educational materials available to help those already diagnosed and those trying to prevent or prolong the disease. While some kidney patients are born with problems, like Izzy, many develop kidney disease through often preventable means.