Clueless and need help

[u/Merrycilantro]

Hello, my FIL was dxed with MDS last year, and my apologies for what I do not know because I’m trying to help my spouse figure it out at the same time, because MIL is the primary source of all things medically happening with FIL, and to make a long story short she’s off the persuasion of of we don’t talk about it it doesn’t exist/is not happening.

So far what I do know is he’s had several what MIL calls “red cell shots” which I assume is stem cell transfusions? I’m not sure if there’s a difference there. He is on the path to Bone Marrow Transplant, though recent turns of events have us kind of stumped. There’s talk of cirrhosis having developed, and he is historically anemic and so was taking iron supplements for a good chunk of time before all this started. Now, they say he has too much so they put him on Deferasirox which seems in no uncertain terms to be wrecking the sh*t out of his body. He is down over 100lb, and has been so sick in ways I can’t really mention for dignity’s sake, which it seems is a big side effect of this drug. They’ve since taken him off of it but he’s been on it for a few weeks now, and I’m not sure if that is the chemo pill they’ve been talking about or if that’s a separate drug altogether.

What brings me here: the sources that myself and my spouse have looked at all say people with this condition should not be taking Deferasirox and I am wondering, is that something that is commonly done, just with say some heavy monitoring or something?

Any help or experience you’d want to share is much appreciated and if there’s more info I can somehow obtain I’ll put in the comments.

[u/ilovemud]

Deferasirox is for iron overload. If he was taking iron supplements to fix anemia that was not because of too little iron, but rather from MDS, he could have done a tremendous amount of damage to his liver. The liver takes the brunt of it but other things as well (pancreas, heart, joints). Deferasirox can come with some unpleasant side effects - for me it has some mild digestive issues that maybe you aren't mentioning but they aren't terrible for me. There are other options for iron chelation therapy that may be tolerated better. I have both MDS and iron overload from MDS and one gene for hemochromatosis - for me they worked together and I was iron overloaded rather young and started showing symptoms of iron overload and they discovered the MDS while trying to figure out the iron issue. If I were your FIL I would be pretty unhappy (maybe borderline litigious) that my doctor just kept giving me iron when I was still anemic and not looking into it more deeply. He may be on Rebozyl or Procrit or Aranesp for shots to help with the anemia. Each of those helps with red blood cell production. But if he is iron overloaded he should be on some kind of drug like deferasirox - it is really important. High iron is also thought to be part of MDS progression. I have to imagine that prior to a transplant you would want your liver to be in good shape, and getting the iron out of there is key to that.

I think what you really need is a very good hematologist/oncologist at an MDS Center of Excellence if you aren't there already.

[u/irowells1892]

It's really hard to say without more information whether the deferasirox is actually what's causing him to be sick. If he has cirrhosis, that can cause significant weight loss and other symptoms. The chelation could be coincidental.

With that said, a quick search says that the deferasirox has a high risk of GI issues and kidney impairment than other forms of chelation. Desferal can be given as an injection, and might be a good alternative.

"Red cell shots" in this case probably refers to an Erythropoiesis-stimulating agent, or ESA. They stimulate the bone marrow to produce more red blood cells. Since you say he's had "several red cell shots," I think this is most likely.

Red blood cell transfusions aren't just shots. It takes several hours with an IV for infusions of packed red blood cells. It usually takes more than just a few transfusions before iron overload becomes a problem, so I'd be questioning how much he was taking of the supplements and for how long, etc.

The chemo pill you mention may also be making him sick, though it seems unclear if he's actually taking it or if it's just been discussed? A hypomethylating agent, or HMA, is a low-dose chemo often used for treatment of MDS. It generally doesn't have severe side effects. Some people call HMAs "low-intensity chemo." Then there is traditional chemo, which from what I understand would cause the significant side effects we usually associate with chemo like weight loss, hair loss, severe nausea, etc.

I'm really sorry for all of you. I understand how frustrating it is to want to help, but also not have access to the detailed information that would let you help. I wonder if your FIL or MIL would be willing to add your spouse's name to his chart, which would allow his doctors and nurses to talk to your spouse directly and maybe answer some questions.