When there is 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 on-call 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. 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 in 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.
At that point, six months post diagnosis, Wilson was taking full doses of Prednisone, Tacrolimus and Cellcept, three stand alone courses of medication used to treat Nephrotic Syndrome. Luckily, adding the Cellcept did the trick. The combination of all three medications working together put Wilson into remission, where he has been for more than two years now.
“We were so relieved to finally identify the right cocktail of medications that worked for Wilson, that are enabling his kidneys to keep working and allowing him to lead a normal lifestyle,” says Andi. “What’s been tough for us this year has been managing the side effects of that. It’s a daily challenge to keep him from getting sick from the common cold to the flu, so that he can stay in remission.” For the past two years, each year Wilson has missed more than 50 days of school. In 2014, Wilson caught a cold the third day of school and couldn’t fight it. It led to a year long infection that would not respond to antibiotics despite how strong. Finally, Wilson underwent four hours of sinus surgery at UCSF to remove the infection.
“One of the burdens families bear, is that for a condition that relapses when a child gets sick, it is very hard to deal with trying to prevent children for children getting sick, because it’s actually quite normal for a child to get sick,” adds Dr. Brakeman. “An average child has 3-5 viral infections a year.”
If anyone in the Callaway family is sick, they have to quarantine both kids. If their seven-year-old daughter Lila has a cold, she has to go into her room and can’t be in the common areas. If anyone in the family has the flu or strep throat, Andi sends them to stay with extended family like her husband’s parents. The consequences of Wilson contracting one of these illnesses and triggering a relapse is too high.
Nephrotic Syndrome impacts the entire family because beyond keeping Wilson healthy and on top of all his daily medications, there is the emotional side as well. Tucker and Andi take special care to make sure their daughter Lila feels loved as there is so much attention put on Wilson.
The Callaways do their best to make both kids feel normal, despite the dietary changes (Wilson’s entire diet is now gluten and dairy free) and the constant hand washing both kids must do. Andi and Tucker have spent a great deal of time educating their school community on Nephrotic Syndrome through outreach and classroom talks. As a result, her kids’ teachers now encourage all of their classmates to clean hands before every snack and before coming in from recess.
Andi doesn’t do carpools the same way as other people, and their kids don’t take the bus anymore to school. They used to, but it’s something they can’t entertain now – it’s just too much exposure to risk. And when it comes to play dates, they’ll only entertain them with kids whose entire family is healthy. If say the child’s sister or Dad is sick, that friend could be carrying something and be unaware, and since viruses typically run through an entire family the risk is there.
This is challenging stuff for a kid like Wilson who loves to hang out with his buddies, doing normal nine-year-old boy things that come along with just being a kid.
“Wilson’s a great kid,” adds Tucker. “He’s always been what we refer to as an old soul. He’s always been a very thoughtful, considerate kid with a great sense of humor. He’s passionate about science, chess, rocks, reading. But his friends and sister Lila top the list.”
Andi and Tucker briefly considered homeschool for Wilson, to have more control of his health. And while they support those that do decide to homeschool, it’s not what they wanted for their family. The tradeoff? Constant planning and strategizing for Wilson’s day before he leaves the house on how to keep him–and his sister–healthy. “Wilson’s condition is on my mind 100 percent of the time, 24 hours a day, every day,” says Andi.
The doctors have told the Callaways they believe Wilson will be a healthy adult. They believe he will grow out of this, and at some point in his life, he will not need to take medication anymore. That is based on the diagnosis of Minimal Change Disease, a category of kidney diseases within Nephrotic Syndrome. “We hang on to that every day,” notes Andi. “It is our hope that eventually Wilson will be a happy, healthy adult. And we try to make every decision, with that in mind.”
Tucker says the most amazing thing he’s seen through all of this is the power of a mother’s love for her child. “I don’t think there’s any stronger force in the world. It’s just inspiring.”
Since Wilson’s diagnosis, Andi and her family have dedicated themselves to changing the story for Wilson and all kids with Nephrotic Syndrome. Supported by their Danville community, close friends and family, they have joined forces with Kara Jones and Jen MacGougan, two other Bay Area moms with the same passion to deliver a cure. Together, these women have been instrumental in raising awareness and funds for the fight in the Bay Area.
On May 21st, Nephcure Kidney International is bringing its annual fundraiser, the Bay Area Nephcure Walk, to our own backyard in Danville. Historically, the Walk has been in San Jose, but after the incredible support from our Danville community last year, this year it will take place at Oak Hill Park in Danville. For more details on the Walk go to http://give.nephcure.org/BayArea.
Last year, the Bay Area NephCure Walk raised more than $100,000, a record setting number almost tripling the year before. As a result, NephCure has announced and begun plans for a dedicated NephCure Accelerating Cures Institute locally at Stanford.
This year, Andi and Tucker have an even bigger vision for this fight and hope to help the Walk top last year and raise $120,000. If you would like to donate to Team Callaway and help support their goal of raising $40,000, you can do so here at http://give.nephcure.org/BayArea/walkforwilson. Donations made to Team Callaway and / or to NephCure go directly toward finding a cure for Nephrotic Syndrome, development of the Bay Area NACI site, and to pay for programming and support for children and families in our local community who have Nephrotic Syndrome. To see other families’ stories, you can visit the Nephcure site here: nephcure.org/connect/patient-stories/.
Want to know more or help? Contact Andi at firstname.lastname@example.org.
NephCure Kidney International is a registered non-profit 501(c)3 and is the only organization dedicated to seeking the cause of Nephrotic Syndrome, improving treatment options and searching for a cure.