Common Nephrotic Syndrome Medications Explained

There's a toolbox of drugs that nephrologists will often pull from when treating nephrotic syndrome. As a patient, I have observed a lot of misunderstanding and confusion among these drugs, so I thought I'd provide a brief explainer!

1) Prednisone -- Corticosteroid

Prednisone tends to be the first line of treatment a nephrologist prescribes because it 1) is often successful in helping a nephrotic syndrome patient reach remission quickly and 2) it's cheap, so insurance is fast to approve it. It's a steroid that can cause a host of annoying side effects, from insomnia to strange fat deposits to excess hair growth to acne to cravings to thin/bruising skin to intense mood swings to anxiety to a heavy chest to a weird condition called "moon face" where your face puffs up's a roller coaster of an experience that's to say the least.

My advice to those prescribed prednisone is to set a limit. If you're not nearing remission in a month or two and you're not having a great time, self-advocate and let your nephrologist know you'd like to try another option. Since prednisone functions like artificial cortisol, your body shuts down its own production of cortisol. Hence, it takes a while to taper off of prednisone since your adrenals need to adjust back to producing cortisol. Therefore, factor that taper time into your decision for how long to try prednisone.

My Experience: the side effects from both the drug and tapering were not worth it for me. I was on prednisone twice and it brought my proteinuria down but by no means enough. (Granted, I have a rare variant of NS that is more stubborn toward drug treatments, but that's a story for another post!)

2) Tacrolimus -- Immunosuppressant

This drug (often sold under brand name Prograf) is commonly used to prevent organ rejection in transplant patients, but it can also be an effective treatment for nephrotic syndrome patients. A lot of people fare well on this drug and it can work in a matter of days or weeks, but many do experience frequent headaches or fatigue. The good news is you don't need to carry out a long taper with tacrolimus, so if the medication isn't working for you, you can explore another option fairly quickly.

My Experience: I had frequent migraines while taking tacrolimus and thus could not tolerate the drug. It also did not help with my proteinuria.

3) Cyclosporine -- Immunosuppressant

Cyclosporine (not to be confused with Cytoxan (cyclophosphamide)'s not) is considered the "older" version of tacrolimus. It works in a similar way to tacrolimus, so nephrologists may consider the two drugs interchangeable. But everyone is different, and some people may respond to one drug but not the other. I responded well to cyclosporine but not tacrolimus...and I've heard many stories of patients who have had the opposite experience. Some people experience cyclosporine side effects like nausea/stomach upset, extra hair growth and bleeding gums. I've been on cyclosporine for almost a year now and my only noticeable side effect is that my fingers and toes are a little sensitive to temperature changes.

My Experience: I respond partially to cyclosporine and it's a great temporary treatment to hold me steady while I explore root-cause medicine (also a story for another post!).

4) CellCept -- Immunosuppressant

CellCept (mycophenolate mofetil) is also an immunosuppressive drug used to prevent organ rejection in transplant patients. It's yet another treatment a nephrotic syndrome patient can try if one of the other options isn't working or sitting well. While taking CellCept, I did not experience any side effects that I could determine were caused by the drug, itself. I've read a lot of other patient experiences that mentioned no side effects...and that it also worked well for their proteinuria!

My Experience: I did not respond to CellCept in regards to my proteinuria. I also had an itchy/painful lip rash called angular cheilitis that came and left when I went on and then respectively off the drug. I can't say the CellCept caused the rash, but it did correlate. I've never heard of anyone else having that rash while on CellCept.

5) Rituxan/Rituximab -- Antibody Therapy

First off, rituximab is NOT chemotherapy. Yes, it is an infusion drug, but it is not to be confused with chemotherapy. It works by depleting your body of B cells (a type of lymphocyte white blood cell), which in turn helps suppress your immune system. This drug is usually one of the latter options tried by nephrologists because it is the most expensive (upwards of $20,000 a dose), and it's hard to convince insurance to cover the couple doses you'll need unless you present a strong case. However, I've heard countless patient success stories about how rituximab was the drug to finally help them reach and maintain remission for a while with minimal side effects.

My Experience: I was barely into my first infusion when I had an allergic reaction. I had full-body hives, itchy ears and a dry/itchy/tight throat. After pausing and starting again, the reaction continued so I couldn't try this treatment any further.

There are other medications nephrologists will also explore like Acthar (similar to prednisone in terms of side-effect profile) and Cytoxan (brand name for cyclophosphamide, a chemotherapy drug that is not used as frequently these days due to harsh side effects like infertility). I've personally never tried these options, and my nephrologist hasn't recommended them to me.

The drug called Sparsentan is currently being tested in clinical trials for nephrotic syndrome patients. If it bodes well, it could potentially be the first FDA-approved treatment for nephrotic syndrome patients.

It's important to note that nephrotic syndrome is a very different experience for each individual. It's an umbrella term for a handful of disease classifications (e.g. Minimal Change, FSGS, Iga Nephropathy, Membranous Nephropathy, etc.) that are poorly understood...and even each disease classification hosts patients with vastly different experiences and symptoms. Truth is there are likely a lot more nephrotic-syndrome causing diseases than current research has been able to explain.

Some people respond quickly to the above drugs and can move on with their life. And boy, that is awesome! Some people have nephrotic syndrome that progresses very quickly, and the above drugs are a literal lifesaver by preserving kidney function.

As for me and my super rare variant of nephrotic syndrome (my biopsy literally says highly unusual if nephrotic syndrome itself isn't rare enough!)...I see Rx medications as a helpful bandaid. I'm blessed that cyclosporine is holding me steady for the time being. But these drugs aren't my shot at remission. I work with a root-cause practitioner who has helped me make big leaps in my journey toward remission...and as the path continues to unfold I'll let you know if I do figure out my true root cause. I think I am close <3

Welcome to Neph Nicki!

I'm so glad that you're here. My hope is that this blog leaves you inspired to keep pursuing your journey toward being your healthiest self, finding what makes you feel happy and pursuing what you envision as living your best life! Hugs ~ Nicki  

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