Story shared by Jessica
My name is Jessica and I'm Addison's mom. I am a medical assistant, and we live near Little Rock, Arkansas. My daughter Addison is 11 years old, and was diagnosed with a form of C3 glomerulopathy (C3G) called dense deposit disease in August 2018, 2 days after her 11th birthday.
It was May of that year when I started noticing her eyes were getting puffy. The other signs were subtle. She didn't want to eat, and she was getting more and more tired. I thought maybe it was just allergies or a bug she couldn't get over. Then I thought maybe it was just a normal part of being 10—growing pains of some sort. But after a while, I started to get concerned. Her tiredness and lack of appetite kept getting worse.
Finally, around mid-July, I started really watching her. She had bad swelling in her legs that eventually moved up to her knees. I knew it wasn't her weight. I Googled her symptoms—which I know you're not supposed to do—but I just knew something was wrong and I didn't know what it was. I took her to the pediatrician on her 11th birthday, which she wasn't happy about, but it was my day off. They sent us for lab work and 2 days later they called us back, but they wouldn't give us results over the phone.
They just said, “Go straight to the Children's Hospital and check in at the emergency room. The nephrologist knows you're coming.”
Based on that, we knew it was a problem with her kidneys, but we had no other details. When we got to the emergency room (ER), her blood pressure was really high and lab work was not normal. We spent the day and night in the ER. Initially, everyone kept thinking it may be strep or an infection that had spread throughout her body. They also mentioned that it could be an autoimmune condition like lupus. They asked if she had taken too much ibuprofen recently, as sometimes medications can injure the kidneys, but she hadn't. Test results were unclear and they needed a better understanding of what was happening in the kidneys.
That's when they went ahead and did a biopsy of her kidneys because they weren't really sure what it was. As a parent, I was nervous because at this point it felt like it could be something really simple or it could be something really complicated. We were trying our best to stay calm, but Addison was nervous. Her first surgery ever was that kidney biopsy. She was a fairly healthy kid. She had never been in the hospital before. It was nerve-racking; we just didn't know how long we were going to be there. There were so many unknowns.
They ended up doing a “rush-read” on her kidney biopsy. A few days later, we got the results and found out it was C3 glomerulopathy (C3G), a rare disease affecting the kidneys. Every sign was pointing to that diagnosis. We were fortunate that the pathologist reading her biopsy had studied this disease for many years and was certain that's what it was. So our nephrologist was confident in what he said.
We were so relieved at first to finally have an answer. But then the nephrologist told us that it's very rare, they don't know much about it, and they're still doing studies to learn about it. That made me even more nervous because even the doctors didn't know much and there wasn't a standard plan of what works. It's been a “let's try this and see if it works and she may get worse before she gets better” kind of thing. It was hard to process because in my experience, when the doctor gives you a diagnosis, the doctor has a treatment plan. But in Addison's case, there really wasn't a clear-cut plan.
A few days later, we got results back that her diagnosis was actually dense deposit disease (DDD), which we were told is a type of C3G. They explained that C3G is a problem with part of the immune system known as the alternative pathway. We tried to explain it to Addison, but that was quite difficult because we didn't really understand it ourselves. Then her physician explained it to her. He told her, “Your kidneys have gunk in them and are not filtering the way they should. Your body is attacking the kidneys, so you have to take medication to help your body to stop attacking your kidneys.”
Once he explained it, she understood as well as an 11 year old could. But she still doesn't fully understand what this means for her future.
At the hospital, the doctors got Addison started on a treatment plan. They started her on blood pressure medicine because her blood pressure was really high, as well as a diuretic for the edema. They were worried about her kidneys, so she had to get a port inserted and got a week of dialysis to flush them out. Thankfully, she responded very well to that. She also got a transfusion. Luckily, they took out the dialysis port before we went home. When all was said and done, we had spent a total of 2 weeks in the hospital. It was a lot to take in. It was a lot for an 11 year old to go through, too—everything from the biopsy and dialysis port to all of the medications.
By the time they finally discharged us, they had started Addison on multiple immunosuppressive drugs and other oral prescription medications to take at home. She got several vitamins and over-the-counter medicines to take every day, too. There were so many that we weren't sure exactly what was working and what wasn't.
She was put on a strict diet. She was restricted to low-sodium foods, limited to 1 serving of dairy a day, and wasn't permitted to have any chocolate. It was interesting trying to meal plan for a while. But now that it's more under control, she can pretty much eat what she wants for now. We do still try to stick with a low-sodium diet to keep her swelling down.
This all happened in August, so Addison missed the first week of school because she had to get used to the immunosuppressive drugs before being back in a school environment. She is a very smart kid and even though she has missed school days this year, she is still at the top of her class. Her teacher doesn't worry about her missing assignments.
Her friends understand that she is still sick and everyone around her at home and school are really great about making accommodations for her. The school nurse lets me know when kids are sick at school so that she can try to avoid exposing herself to colds and other viruses, and they check on Addison, especially for any swelling. Her day-to-day life is a little different now. She meets with her doctor every 2 weeks for a checkup. I talk to her physician and the school nurse once a week. We have to monitor her blood pressure and weight. She has to carry a water bottle and hand sanitizer with her.
Luckily, Addison has adjusted really well to her new routine, but at first it was really hard—not being at school around friends and having to take it easy with volleyball, which is her absolute favorite sport. But my husband and I both agreed that we are not holding her back. If she wants to do something, we’re letting her do it. She knows when to take a break. She does the best she can to adjust to it.
She's very good about staying on top of what she needs to do. Every day, she wakes up, weighs herself, eats breakfast, takes her medicine, takes 1 medicine at school at noon, and takes her iron pill when she gets home from school. She is very aware and is great about remembering everything. At night, we eat supper and she takes her night meds. We also take her blood pressure at night. Then she goes to bed. We know what needs to be done day to day. It's the new normal. We don't really think much about it; it's just what we do.
Addison has her good days and bad days, like when she doesn't want to take her medication. But I always tell her, “You have to just take things one day at a time. Things will get better.” So for now, we're taking it day by day to figure out what works and what doesn't work.
This whole experience has taught me a lot—not only about this disease, but also about my daughter and about strength and resiliency. We've learned as a family to always try and stay positive. I can't dwell on the “whys.” We just take things a day at a time and try to let her be a kid. This diagnosis turned our whole world upside down, but we have learned not to take things for granted and to appreciate the little things in life. It took some time to come to terms with everything but I think even in a few short months, we've finally found our new normal.
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