Hannah G.

Thank you for taking the time to listen to Hannah’s story.

We found out by accident (she had neck pain that sent us to the ER) where they found she had a high SED rate, which led to a urine test that showed a lot of protein in her urine. That led to her doctor setting us up with a nephrologist who decided to do a kidney biopsy that showed my daughter has FSGS kidney disease. She also has nephrotic syndrome.

She was 6 years old at the time of diagnosis and she is now 9, and so far, nothing we have done has helped put her into remission.

From there, we found out she has many other issues by doing bloodwork. We have been to 4 children’s hospitals, and nobody can give us answers—they just say her case is rare.

She has low IgG and is immune compromised, high IgM, high SED rate, proteinuria, enlarged spleen, enlarged thymus, bronchitis, right middle lobe syndrome, pneumonia several times a year, FSGS kidney disease, NS, bloody noses that cause large clots she coughs up (this has not happened in a long time), leg and foot pain, bad itching episodes—and this isn’t even everything.

She sees nearly every specialist such as genetics, nephrology, rheumatology, immunology, neurology, and pulmonology. In 3 years and many, many tests later, we are still no further than when we started with answers.

Her nephrologist (kidney doctor) was trying to lessen the amount of protein she was spilling in her urine, as some kids could go into remission from FSGS with steroids. She was 53 lbs at the time, and they put her on 50mg of steroids daily for 8 weeks.

I wanted to call quits at week 5 because I could see everything getting worse. She had things that happened that she didn’t have before steroids such as:

• high blood pressure
• cholesterol levels spiked
• high sugar
• her liver became enlarged
• stomach pain and bloating
• moon face
• swelling all over
• her urine turned milky looking
• tremors
• lethargy
• hunger
• 12-pound weight gain
• heart palpitations
  and more.

It was awful. That taught me to always stick with my gut instinct, and it’s okay to say no.

Not to mention, her protein went from 4,000 to over 17,000 on steroids. As we weaned her off, thank goodness everything went back to how it was before—no more bloating, swelling was gone, no more moon face, urine color returned to normal, etc.

She was deemed steroid resistant. Her protein is still between 4,000–11,000, and it’s constantly going up and down in between those numbers. One day it can be 7,000 and the next day it can be 11,000, which I do not understand how it can fluctuate so much.

So, we know we have to try something else in hopes we can preserve her kidneys as long as possible. I will attach a photo of her before steroids and then on steroids (picture of her swollen sitting on her bed is on steroids). She never looked like that until we started steroids. Thank goodness once she weaned off she went back to her normal self.

We know this much protein will cause her kidneys to decline faster and that scares us to no end. Her nephrologist has talked about trying immune suppressants such as Cellcept, Tacrolimus, and Rituximab to list a few.

And just like steroids, it’s possible that these medications do not help her protein at all and can give so many scary side effects or make things worse.

The odd thing is that all of these options to treat her protein spilling—including steroids—are not kidney friendly, which is so odd to me. Her IgG levels are low and putting her on an immune suppressant when she’s already immune compromised is scary.

She gets sick and hospitalized with pneumonia at least yearly and almost had to be air-lifted to a different children’s hospital. Instead, they ended up sending an ambulance to the hospital we were at around 2am to transfer her to a better children’s hospital PICU 3 hours away. It was scary, to say the least.

I feel like throwing my hands up in fear that all of these drugs can cause more harm like the steroids did—and in the end, to find out she’s steroid resistant.

Some of the immune suppressants talked about increased risk of cancers, brain, and lung problems, among other things. I feel like we are trying to fix one issue, but it could cause another issue that’s maybe worse. I feel like they are just guessing on treatment because nobody knows what’s driving all of these issues.

I feel like she’s a human guinea pig with not really knowing what to do but just picking a medication to start… it’s a try-it-and-hope thing.

For example, with diabetes you know the drug that is needed to treat it. With FSGS, that’s not the case.

I just don’t want her going through such harsh side effects or making the wrong decision for her and me having regrets that I’m not doing what is right.

Also, it’s not only FSGS that she has but a bunch of other things listed above, and they are unsure why.

They do not even know what is causing FSGS other than they ruled out that it’s not genetic after her genetic doctor did extensive genetic testing including a full exome.

She has been on Enpaned for 3 years now with no change, and we started her on Tacrolimus 3 months ago with no change.

As Hannah’s mom, I’m always in fear that something bad is going to happen to her or her life will not be as long as it should be due to FSGS.

I know it bothers my daughter too, but she never shows it. She often asks me if her kidneys are doing better and healing on her new medicine.

As a child, she thinks she takes medicine and she will be all better. As a mom, how do I dare tell her no, they are not getting better? I don’t have the heart to do that.

I just tell her that we are still working hard to get her protein spilling under control so her kidneys will be happier.

We feel, along with several of her specialists, that once we can get her kidneys into remission, a lot of the other issues—such as low IgG—will go away.

My husband and I definitely live in fear. My husband has kidney issues as well, but it has been determined that his kidney issues are not related to my daughter’s kidney issues.

We are just frustrated that this many years later and nothing has been done to help my daughter. I don’t want her to feel different than other kids.

We are doing bloodwork and urine often, and I know she knows that’s not normal.

My apologies for going on and on about Hannah’s story and being all over the place while explaining her journey.

To be honest, I don’t even think I have listed everything, but I just pray and hope we can get Hannah into remission.

I stay up into the early morning hours doing my research and talking with other Mamas in the FSGS group—and sometimes crying when I think about Hannah’s future.

She deserves everything, and I pray one day we can find a cure for FSGS kidney disease. Thank you for taking the time to listen to our story.