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AliveMay2016

A Family’s Courage in the Face of “No Known Cause or Cure” Nephrotic Syndrome You really never know what life has in store for you...until there it is —in store for you. One fall day, three years ago, Tucker and Andi Callaway found out just that. Their first child, Wilson, woke up for kindergarten like any other Monday. But that day his eyes were just a slight bit puffy. Andi, Wilson’s Mom, didn’t think much of it, but she kept him home from school. As the day went on, he seemed fine, and the next day, despite puffy eyes again, no other symptoms. Thinking it might be an allergy, Andi changed the sheets, switched detergents and rewashed. But after a couple of days went by, she knew there was something wrong. Still, even a visit to the oncall doctor didn’t leave Andi or her husband Tucker with answers, or with any cause for concern. Despite knowing something was brewing, Sunday the family enjoyed a trip to the Lawrence Hall of Science and stopped off for a special occasion lunch of a burger and fries. “By the end of the day that Sunday, his legs were swollen and as big as mine,” says Tucker, Wilson’s father. “They were like liquid deformities on his body. We of course rushed him to the emergency room.” “It was crazy,” says Wilson, who is now a third grader at Green Valley Elementary. “I felt like I had all this extra fluid and my face was really swollen and stuff.” Andi remembers thinking when they told them it was Nephrotic Syndrome, something she and Tucker had never heard of before, that it didn’t seem that bad. “It seemed like a good outcome, considering all the bad things so many kids are faced with. It wasn’t until the weeks and months went by that we really knew what was in store for us,” says Andi. As it turns out, Nephrotic Syndrome is, unfortunately, bad. It is a chronic disease that has no known cause and no cure. It is not genetic and no two cases are the same. It affects boys more than girls, and each year 2,400 children are diagnosed with it. Adults can be affected too. What is known is that Nephrotic Syndrome is a serious non-contagious autoimmune condition that prevents the kidneys from properly filtering blood. In normal function, the body responds to an immune system trigger like a cold, virus or bug bite, and then shuts off when the trigger has passed. In Nephrotic Syndrome, the immune system attacks the trigger, but then turns and attacks the kidneys causing them to become inflamed. As a result, the kidneys can’t keep the right balance of protein and liquid inside the bloodstream, so liquid seeps out from the bloodstream and into the tissues around it. “It’s a mystery—we don’t really know what causes it,” says Dr. A L I V E E A S T B A Y m a y 2 0 1 6 Paul Brakeman, Assistant Professor of Pediatrics and Pediatric Kidney Specialist, UCSF Children’s Hospital and Wilson’s Nephrologist. “We have medicines we can use to suppress the immune system, not to cure it, but suppress it and put people into remission. But we don’t really have medicines that get to the exact heart of what causes it, because we don’t know what the cause is.” That is the challenge with Nephrotic Syndrome—there is no standard protocol for treating these cases because there is no standard case. Initially, Nephrotic Syndrome is treated with high doses of prednisone for three months and a hope that the kidneys respond and the patient goes into remission. If all goes well, they will remain in remission after the three months of treatment is complete. In the attempt to treat Wilson, doctors prescribed a daily dose of 60 mgs of Prednisone. If you have taken even 5 mgs of prednisone, you can imagine what a toll 60 mgs will take on a six-year-old child. Prednisone is a miracle drug; but it comes with a price. It is extremely toxic to the body and has severe effects on behavior. “There would be days when Wilson would throw a temper tantrum for hours—literally hours,” says Andi. “Life was totally unpredictable, and while we were grateful for the medicine that put Wilson into remission, our whole family bore the burden of that treatment.” And then came the rest. The next six months brought one relapse after another. “We had finally pulled our family together after his diagnosis and we thought, ‘Okay, we’ve got this. We can handle Nephrotic Syndrome.’ And then Wilson relapsed before completing his first course of prednisone,” says Andi. So they started again. Another three months of prednisone. “But just when we finally regained our sense of hope, Wilson relapsed again,” explains Andi. What do doctors do when the initial treatment isn’t working? They prescribe second line medications—medications that are designed to spare the body the toxic effects of long term steroid use. In Wilson’s case, they combined the Prednisone with Tacrolimus, an immune-supressive drug used mainly after organ transplant to lower the risk of organ rejection, with a side effect linked to cancer. In fact, the cancer risk is considered high enough that in a hospital setting, the administering person must wear gloves when giving the dose. But that combination also failed as Wilson relapsed twice while taking those two drugs. The doctors finally added Cellcept, another immunosuppressant medicine similar to Tacrolimus with another set of potential side effects including asthma, respiratory tract infections, anxiety and again, cancer. 18 E L ENA ARNE Y


AliveMay2016
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