Early February marked five years since I was diagnosed with this disease. March will be six years since I first began having symptoms. On one hand, it’s hard to remember a time when this disease didn’t dominate my life. On the other, I’m so grateful that I’m doing as well as I am and that I’m not on dialysis.
February is also “Rare Disease Month,” so this month is a two-fer for me. Rare Disease Day is February 28th. Throw a big old party! Celebrate that you’re not part of the club!! My disease is so rare it’s not even listed in the National Organization of Rare Disease’s database. A rare disease is defined as affecting fewer than 200,000 people in the US. I was last told there were 400 known cases of my disease in the world. I’m sure that number has increased but my last message to my former nephrologist in Pennsylvania asking for an update on the prevalence went unanswered. And it’s still kind of mind-blowing that I’m the only known person receiving the treatment protocol I’m on.
Well, sorta. I read an article about a man with the same disease I have and something called light chain proximal tubulopathy, another rare kidney disease. Per the article, “Their coexistence is exceedingly rare.” I mean, I thought I was unlucky. He was treated with Velcade, which I was on until I started getting the side effect of neuropathy, cyclophosphamide, and dexamethasone. This drug regimen was successful, and he is in remission. They’re not the exact same drugs I’m on but close. From what I understand, Kyprolis and Velcade are very similar medications. I’m not on cyclophosphamide but hey, I’ll keep it in my back pocket if what I’m on stops working.
I had a bunch of lab work done about a month ago. To my utter surprise, EVERYTHING came back normal. My A1C was 5.4. That was shocking! My lipid panel was normal. Last time, about a year ago, my triglycerides were pretty high. They dropped from 190 to 64! But I was unaware that high blood sugar will increase triglycerides. The endocrinologist told me that if I had taken steroids within about 10 days of the test, they would have increased my blood sugar and therefore the triglycerides. At that time I was on chemo (and also steroids) at least twice a month and possibly three times a month. There’s no doubt I would have taken them within 10 days of the test. Why didn’t anyone tell me that?? That is so frustrating.
My endocrinologist is a very good doctor. She’s got a lot of information and knowledge about things that I in my pea-brain would not expect to fall into the scope of endocrinology. She is also a hoot. I described her as such to a doctor today who knows her, and she said, “That’s about right.” We had a video appointment in early January, and she said with regard to my lab results, “You’re just taking SUCH good care of your health!” I told her, “Well, no, I’m really not. I don’t really do anything right, except drink a lot of water. I don’t get enough sleep, I work too much, I don’t eat right, and I don’t exercise.” “Well, I think you need to accept that maybe you’re taking better care of yourself than you think.” I think it’s the combination of medications I’m on that are finally getting things under control. I appreciate her vote of confidence though. And I definitely appreciate the fact that she’s got me on the right path with these meds.
I had another 24-hour urine test earlier this month. This is to serve as our ‘baseline’ since we’re decreasing the frequency of my chemo. And once again, there was NO measurable protein in my urine. This has now been the case since March 2023. Two years! Wow. That’s progress.
I was supposed to have chemo today but Monday I had a call from one of the nurses saying that, once again, my insurance company is denying my treatment so they had to cancel my appointment. I guess this was to be expected because it had been approved through January. I don’t know what happened a few months ago when my doc said they had already started denying the claims even though it HAD been approved through January. Anyway, my doc has a “peer to peer” meeting tomorrow with a doc at my insurance company, and hopefully I will get approved again. If not, I’m not sure what the next step is. I know the company who makes Kyprolis has some sort of “patient-assist” program for patients who don’t have coverage for the drug. I was given some limited information about this quite a while back when they denied coverage. But I was never given specifics because my insurance changed their mind and decided to cover it. I have no idea how the program works or how much I would have to pay.
But god. As of this year, my out-of-pocket cost is $7500.00. That’s what I have to pay before my responsibility is met. This is up $1000 from last year. Last year I met that cost by the end of April. This year it will probably take longer since the chemo is only once a month.
As I’m sure I’ve mentioned before, I live on a budget. All my spending is accounted for. So right now, each month I have to put away $792 to cover my part of the plan, plus the $2000 I’m opting to pay to see my endocrinologist who doesn’t take insurance. So…$9500 a year. This is in addition to what I pay monthly for the privilege of having health insurance at all. And yes, I’m well aware that seeing the ‘concierge’ endocrinologist is a luxury I’m fortunate enough to be able to afford; I don’t take that for granted. I probably won’t have to see her forever, but right now, I don’t feel as though I have a choice.
So if my doctor doesn’t prevail and my chemo isn’t covered, and I have to pay for it, holy crap. I will then be revamping my budget once again. I guess I will even MORE grateful my chemo’s only once a month.
Last week I got a pop up notification on myChart that I had an outstanding balance of $249.00. I went ahead a paid it, even though I have no idea what it was for. Today I got the actual bill in the mail, and it’s an outstanding balance from 2023. What? How does that happen when I had already met my out-of-pocket costs for that year? Maybe at some point in the near future if I’m able to muster up the fortitude to call them and question it, I will. Right now, it just sounds like it will be a very frustrating phone call and waste of my time. But $249 ain’t nothing to sneeze at either.
I had another bone density scan a few weeks ago due to the long-term use of steroids. My previous one in 2021 was normal. Luckily, this one was normal as well. Interestingly, as I was driving to that appointment, I saw a new (or at least new to me) nephrology group. AND they take my insurance. They have several providers too. I’m happy enough with my current nephrologist, but I’m always on the lookout for other providers should any of my docs have the audacity to retire. Most of my providers are through Lovelace, but they do not have any nephrologists on staff. I wonder why.
Back in 2020, I started with a nephrologist here that I liked well enough at first, but we didn’t see eye to eye on my treatment. She didn’t think it was worth pursuing this chemo path I’m on and thought I should resign myself to dialysis. I can understand why she had that opinion. I mean, it was (and is) all experimental. But dialysis will always be there if I need it. I was certainly eager to try just about anything to ward that off for as long as possible. She was also the doc who told me I would have to find a specialist who could help me. That was just wrong. That should have been her job, not mine. So… we parted ways. However, I did like her partner in the practice and would definitely consider seeing him. He did my initial kidney biopsy and was supportive of going the chemo route. Although it might be awkward to see him after I’d already seen her, enough time has passed that it shouldn’t matter. Not that it should matter anyway. This “stepping on toes” crap shouldn’t be an issue regardless. But some doctors have fragile egos. I’ve worked with plenty of them.
I was supposed to see my nephrologist in March. I called about two months ago and was told he was already booked through May, but to call back in a couple weeks to schedule in June. So, I put a reminder in my phone to call again in two weeks. When I did, she told me his schedule for June was booked and that I should have called sooner. I said, “But you told me to wait two weeks and then call.” “Yes, but you didn’t have to wait two weeks. You could have called sooner.” “But YOU told me to wait two weeks before calling because you wouldn’t have his schedule updated before then.” (And it was the same person I had spoken with before. It’s a small office, and I know her name.) “Yes, but you could have called sooner.” Okay, that conversation was going nowhere. So as of now, I don’t have an appointment with him. No biggie. Just frustrating.
Today I had an appointment with the endocrinologist through Lovelace. Although I’m paying to see the ‘concierge’ doc, I still need to stay in the Lovelace loop in the event the other doc retires, moves, wins the lottery, what have you. And she is in agreement with that. But the doctor today couldn’t understand why I was seeing both docs. I explained my reasoning to her but she still said she thought it was unnecessary. I said if the other doc stopped practicing, I couldn’t run the risk of having to wait several months to a year to get established with another doctor. I think she finally just decided she didn’t care and moved on.
She said she started with Lovelace in October but will probably be leaving soon. (See? This is exactly what I’m talking about! Docs leave all the time!) I said, “I don’t know what it is about Lovelace but they sure seem to chew ‘em up and spit ‘em out.” She said, “You hit the nail on the head.” We talked for a bit about the state of medical care in New Mexico, and we both agreed that the average citizen doesn’t understand how critical the situation is here. Unfortunately, we’ll all find out soon enough.
She said I don’t need to come back for a year. Sounds good to me. After the appointment, I read her notes in myChart. “Neck is supple, no lymphadenopathy.” She must have diagnosed that telepathically because she never came within 5 feet of me. She certainly didn’t feel my neck. “Lab results reviewed and discussed in detail with patient.” Nope. Didn’t happen. “Patient was advised to follow a healthy diet, get regular exercise, and practice good sleep hygiene.” Nope, nope, and nope. None of that was discussed. Oh well. What’s a little falsification of medical records among friends, right??
I’ll see the concierge doc in March. She is going to change my meds from Ozempic to Mounjaro. For whatever reason, she feels the need to show me how to use the injection pen. I feel pretty damn certain I could figure it out but whatever… if she wants me to go in, I’ll go in.
I finally came to a realization about my balding head. I had read up on Kyprolis, and that didn’t seem to be the culprit. Why it took me so long to realize this, I don’t know. But it’s the steroids! I googled “steroid use and hair loss,” and BAM. There it was. I should have known. Of course it’s the steroids! It’s ALWAYS the steroids. It’s caused so many other things I’ve had to deal with: cataracts, diabetes, moonface, perpetual thrush, compromised immune system, and, oh, not to mention it almost killed me from congestive heart failure. So why wouldn’t it cause my hair to fall out?? The frustrating thing is that none of my doctors have told me this. I mean, what the hell.
I mentioned before that I didn’t realize how strong dexamethasone is. With the decrease in my chemo schedule, I’m now only taking 12 mg once a month. Way back in the beginning of all this, I was taking 18 mg four times a month. But to me, that didn’t seem like much because before that, I was taking 60 mg of prednisone per day. So 18 or 12 mg just didn’t seem like a lot. WRONG. 12 mg of dexamethasone is equivalent to 80 mg of prednisone, and 18 mg is equivalent to 120 mg of prednisone!! So yeah, it’s a LOT. Enough apparently to cause my hair to fall out!
I have an appointment with the dermatologist who specializes in hair loss at the end of March. I’ll be interested to hear his input. But I don’t want to take any oral meds so I will only consider something topical. And even then I will need to read up on it to see if it’s something I want to pursue. There’s always a wig.
My newest side effect from medications is tinnitus. I’ve had ringing in my right ear for more than 30 years, so it’s rarely bothersome. But now the left ear is joining in, and it’s at a different pitch than the right so it’s really driving me nuts. And it’s just constant. I have a set of hearing aids from a patient who passed away and decided to try them to see if it would help, and it really did! The problem is using them at work because I wear a mask, glasses which are on a chain since I constantly have to take them on and off, and a headset when I’m doing hearing testing. Adding hearing aids to that mess is kind of ridiculous. But I still wear them when it’s really annoying, and I wear them occasionally on the weekends. It does make a difference, for me at least, so it’s nice to know when I tell patients hearing aids will likely help with their tinnitus, I’m not just blowing smoke.
Kidneys in the News!
This is pretty exciting:
FDA greenlights first clinical trials for genetically modified pig kidney transplants in humans
The title says it all, essentially. Two companies will begin testing pig kidneys on a larger scale, starting with six patients then increasing to 50. Patients who are not candidates for human kidney transplantation due to other health conditions or patients who would likely die before a kidney becomes available will be selected.
I had read the first pig kidney recipient had died, unrelated to the transplant, but I didn’t realize the second recipient also died. She received both a pig kidney and heart transplant, but the kidney failed after it was damaged by her heart medications.
A woman from Alabama has now surpassed the two-month mark with a pig kidney, and so far she is doing well. She is only 53 years old and had donated a kidney to her mother in 1999. If anyone deserves this transplant to work, she certainly does!
Good article about dialysis, pig kidneys, and gene edits:
Who wants a pig organ? Patients sick and tired of waiting years for a transplant
Low, Low, Low 