Hi! Remember Me? I’m TSC!

I had another check up Thursday.  My lungs are still doing great,  but I wish I could say the same about my kidneys.

I started back on Rapamune (sirolimus) yesterday, so I’m confident that might help when it fully kicks in, as it did when I was pre-transplant.

I couldn’t have contrast for my kidney MRI because my creatinine ratio & GFR were too high & too low.   Had I done so, it could have caused further irreversible kidney damage.

I’m grateful that my local nephrologist explained to me why these lab values are important & the danger the contrast could pose ahead of time.

Later I learned what is considered a good range for me post transplant because I noticed an upgraded diagnosis on my medicine list & immediately asked questions about it.

I’ve included a link to the National Kidney Foundation (NKF) above so if people are curious they can learn more.

With my Tuberous Sclerosis Complex (TSC) looming in the background, I was expecting this somewhat, but was still taken aback when I found out my values constitute Stage 3 chronic kidney disease, (CKD),  as I had always been at a Stage 1 for several years.

The Renal Association which I just linked to, explains the staging well & it put me more at ease.

The CKD staging was a new learning curve for me,  I’m always learning new things, especially now post transplant.

In my case also, it wasn’t super surprising as I knew my kidneys would take a hit from the immunosuppression drugs too, even outside the issues stemmed from my rare disease(s).

The additional problem of my worsening kidney function is because of both TSC & LAM I have (kidney) angiomyolipomas.

I’ve had them treated before because they’d get very large & prone to bleeding because the tumor blood vessels are very brittle.

They take over healthy kidney tissue & even though they are benign, it doesn’t mean they aren’t problematic.

This time, they were smaller than in the past (even after being off Rapamune [sirolimus] 6 months while my airways were healing), but some of them looked a bit irregular, which can indicate a problem sometimes.

Normally, I’d get them biopsied right away,  but it’s different this time, it’s watch & wait.

Biopsy could make me prone to infection or set off a cascade of rejection.

I’m comfortable with a re-scan in 6 months because they aren’t doing anything, they just look less fatty than normal.

Based on past experience with kidney biopsies of my tumors, they’ve been fine.

If there’s nothing to worry about, we’ll know in 6 months.

If there is, we will also know, then we’ll deal.

I worked too hard for these lungs to risk them right now without concrete evidence of an issue.

Knowing my body as I do, I was adamant about going back on Rapamune (sirolimus) this month because I noticed a fingernail tumor.

These are an often classic hallmark of TSC that can appear on fingers & toenails.  (They are called subungual fibromas.)

When there’s no activity present or when Rapamune (sirolimus) is controlling growth, these aren’t visible & I don’t have them.

They were one of the first things I noticed to go away when I started the drug back in 2009.

Also the tumors on my face got smaller & less red.

I had noticed the past month or two more redness & a few new growths popping up.

When I pointed this out to him, my transplant doctor & I both agreed I needed to go back on.  (Before mentioning that he was hesitant about putting me back on because I was doing so well, he didn’t want to rock the boat.)

I’m glad that if I do challenge one of my doctor’s decisions, I explain why & it’s given equal weight.

In this case, had I not mentioned it, I may have gone at least another month or more without the drug & maybe it wouldn’t have made a difference, yet it just might.  Based on past experience, when my TSC activity is high, I get larger, more problematic tumors.

This is another reason why it’s important to be in tune with my body.

Because lung transplant surgery is such a major shock to the system, & there’s such an adjustment that first year because of tweaking of immunosuppressant meds, checking for signs of rejection, monitoring other common symptoms like nausea to be sure they are managed,  it’s very easy to overlook seemingly smaller things.

But small things in rare disease can become big things very quickly sometimes.

Since it is WorldWide LAM Awareness Month, I know there are friends like myself that have to manage 2 diseases at once & it can be a handful to do so.

I want you to know if you are doing your best to advocate for yourself you are already ahead of the curve.

Just keep doing what you’re doing.   You don’t have to be perfect, you need only be yourself.

Some of these issues can be rough to face, especially if compounded with many issues all at once.

It seems you get one under control, another flares.

It’s hard & exhausting for sure, but all we can do is our best.

Keep rolling with those punches.

You’re making your way!




3 thoughts on “Hi! Remember Me? I’m TSC!

  1. Let’s hope the rapa helps you. I see a nephrologist in September to hopefully go on the drug. I am not able to have embolisation as my kidneys are full of the angios and the surgeon said if he tried to do anything the chance of me being in dialysis will increase 95%. So everything crossed for you xx

    Liked by 2 people

    • Thanks I’m confident it will. It worked great before. We shall see though :). I’m very sorry you can’t have the embolizations they are pain but before rapa it was the only thing that helped me treat those problems. I am hoping for the best for you in sept. You’ll have to update me and let me know how things go if you can.

      Liked by 1 person

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