Day -12: Go Kidneys Go

Zoe had her 3rd GFR this morning, a follow-up on previous tests to determine kidney function. It involves injecting a radioactive fluid into her central line, then testing how long it takes to process. They take two blood levels after a delay, check how much remains in her blood stream, and from that we can tell how well her kidneys are functioning.

Zoe’s last GFR reading was 49, which essentially means she was running on 1/2 kidney function. What we didn’t know is whether that was a temporary problem, a permanent one, or even if it was part of a continuing decline.

Today we know the answer, it was temporary. Her GFR today came back 66, which means she’s regained improved about 30% over where she was. It’s great news, but the most important part is that she is improving, not deteriorating. This would seem to indicate Dr. P was right that the previous GFR results were directly related to her Cyclosporine use. He couldn’t promise that we would see her kidney function return (ever) after stopping Cyclosporine, but now we know that it has.

Go Zoe!

Next week, is a big week, we’ll be doing her last week of pre-conditioning and be admitted in advance of her Stem Cell Transplant. More on that Monday.

Day -14

No big changes today. We went in for a brief clinic visit to do blood counts and vitals, the big news from that is that Zoe’s blood pressure continues to be solid (90/60) off of her BP medicines. This continues to give us hope that her kidneys have or are recovering from her prolonged cyclosporine use. We’ll be doing a new GFR (kidney function test) on Friday, and then we’ll know more.

‘Til then, here’s Zoe playing with her favorite toy.


Day -22: Starting Campath

We’re off and running now. Campath started today at 11:30am on a stepped dosing process, which means she gets an increasing dose every 15 minutes under observation until we reach the full test dose. The test dose will run for several hours, and is 20% of what she will receive the rest of the week.

Zoe was given pre-meds to help her manage the chemo: hydrocortisone (steroid), benadryl (antihistimine), tylenol (fever), and demerol (pain).

She is asleep, no surprise. We’re told to expect hives, rashes and fevers, so we will see how things go.

Zoe’s blood counts brought good news today, something we are grateful for on this day in particular. Her Creatinine, a marker of kidney function, is now down below .1, which is as low as can be measured. Her most recent test on Friday was .2, which means that her kidney function has improved measurably. Go Zoe!

The numbers:

  • WBC: 5.0
  • Hemo: 10.4
  • Sodium: 137
  • Platelets: 667
  • Seg Neutrophil: 38
  • Lymphocyte: 55
  • Creatinine: <0.1
  • Bilirubin: 0.5
  • AST: 37
  • ALT: 20
  • ANC: 1900

These are the numbers we’ll be following most closely from now on. The creatinine as mentioned is a marker of kidney function. Bilirubin is a marker for liver function, and it’s currently perfectly fine.

AST & ALT will be monitored to determine if we need to hold the Hydroxyurea. If they rise to 3x “normal” or mid-range, then we hold it. 3x “normal” is ~180 for AST, ~66 for ALT.

ANC means Absolute Neutrophil Count, which is a somewhat complicated calculation that measures immune system function. We can expect that to go up and down, but mostly down, during conditioning. After Zoe’s transplant this number will become our holy grail. We’ll be looking for her cell counts to go up and her ANC to rise and stay above 500, as I understand it. We’ll learn more about this closer to the transplant.

The campath will quickly reduce the Lymphocytes, which is what it is there to do. The “segs” will remain elevated and the ANC, which counts both, may therefore stay high initially. Eventually we expect it to go way down and not recover until after transplant.

Afternoon Update:

Zoe’s first day test dose went very well. No major reactions today. She had an elevated heart rate and very mild fever for about an hour, but was otherwise fine.

Because Zoe did so well, we will be giving her Dexamethasone for the next 3 days during Campath instead of the normal steroid given. This is because Dexamethasone penetrates the blood/brain barrier more effectively, and with HLH it can do more to suppress any abnormal cell activity than the traditional steroid given with RIC.

Tomorrow we continue the chemo and step up from the today’s test dose (which was 20% of normal strength) to the full dose. We expect if we’re going to see a reaction we should start seeing it tomorrow.

Calm Before the Storm

We’re out of the hospital for a few more days, it’s our last true break before things really wind up. Tuesday is the beginning of Zoe’s pre-transplant conditioning, a process that will take about 3 weeks.

We wrapped up this past Tuesday with a spinal tap and bone marrow aspiration and a final pre-conditioning meet with Dr. P. We went over our questions and all of the test results, and in general the meeting was pretty somber.

It was time to commit. Time to sign papers and stomach all of the dire warnings that come with a procedure such as this. Every known side effect, no matter how rare, every worst case scenario. It can be really disheartening, but there isn’t a way around it.

Zoe’s test results were mixed. The vast majority of her tests results were great. Her kidneys however are still at half strength, which is really concerning. Also of concern was the renewed presence of histiocytes in her spinal fluid, something that we had successfully resolved once before with intrathecal chemotherapy. It only took one shot of it to suppress the histiocytes last time, and our hope is that the same will hold true this time, giving us time to get to conditioning without any problems.

What this tells us with very little doubt is that she has FHL. If she did not, we shouldn’t be seeing that activity any more as I understand it after treating it successfully the first time.

Outside of these two factors we are in pretty good shape, but we are of course stressed at anything that’s not 100% going into conditioning. Neither of these things will necessarily impact Zoe’s chances, however. The disease activity will be remedied very shortly with conditioning, and the kidney function doesn’t effect her risks of infection or GVHD, our worst fears, or her chance to graft.

What it does is raise the risk of fluid retention and future kidney problems, as well as their ability to use certain medications if things take a turn for the worse. This isn’t an uncommon situation though, the transplant team is well versed on handling such details. We’ve already modified her treatment plan slightly to adjust for minimal kidney damage from medication where possible.

At this point we’ve committed to our course. Pretty soon we’ll be almost entirely back in the hands of a medical team. ¬†We have to rely on our efforts to research everything in making our choice, and hope our trust and faith are well placed.

Cyclosporine & Sodium, Updates

It’s Monday and we’re back to the day hospital at Duke. Today was blood draws and a redo of the GFR kidney function test.

On Friday they took a Cyclosporine level and it was really high, 590 or so, which is much too high for Zoe. It’s a bit of a mystery, since we haven’t changed her dose and, after much hand wringing, feel sure that nothing happened to give her too much. We just don’t know how it could be that high. Normal therapeutic range is 150-250.

She did get a dose just before the test was taken, and we’ve seen in the past that that can amp the results up considerably. We got a reading in the 400’s awhile back after she had a dose just before the test, but 590 is just very high.

So, she’s off Cyclosporine, probably for good now since it is not a part of her conditioning regimen.

We retested some levels today along with the GFR, and found that it was still at a therapeutic level, 112, despite being discontinued days ago. Her GFR was not improved, still about 50 (100 is what we want), but the feeling is that the continued presence of Cyclosporine is keeping it down.

On a positive note, her Sodium has gone up to 137. It’s been down throughout her entire treatment, ever since she began her chemo, but we did no have a reason for it. It would now appear that the Cyclosporine was causing low Sodium for Zoe for some reason.

This doesn’t change much for us, we still proceed ahead, but we’ll be monitoring her kidneys via other markers as we go.

Tomorrow is another spinal tap and bone marrow aspiration, another meeting with the transplant team, then home for the week we hope.