New member - father just diagnosed with MDS-EB-2

[Catl]

Hello all,

I have been a lurker for the past few weeks and have finally found the time to post a message. Thanks to everyone for posting messages and sharing information - it has been so helpful!

I am new to the Forum. As you can see from my signature, my father was recently diagnosed with MDS with excess blasts (previously RAEB-2, renamed since new 2016 WHO revision). It has taken forever for him to finally receive a diagnosis and the wait was brutal. We live in Canada and while it is great that we don't have to worry about private insurance, the system is bogged down and we have no choice but to wait (took almost 2 months to receive initial diagnosis). He also received an initial diagnosis of AML based on previous WHO criteria and was transferred to a large hospital to receive IC but the larger hospital re-did another BMB and they upgraded his diagnosis to MDS with excess blasts type 2 and are now recommending Vidaza. He has a total of 15% blasts but the majority of the blasts are erythroblasts (13%) and only 2% being myeoloblasts. He also has 70% erythroid precursors. Under the old WHO classification he would have been diagnosed with Acute erythoid leukemia. It's kind of scary that 6 months ago he would have received IC but that now he will be receiving Vidaza instead. I hope that the hematologists made the right call.

With respect to the Vidaza, I am wondering if anyone can help me with a concern that we have. His blood counts have been very low since Nov. He basically has required HGB transfusions every 7-10 days and has also been having platelet transfusions every 4-5 days for the past month (HGB generally around 80 or less and platelets have gone as low as 6 (generally between 10-19). I read that with Vidaza, it can get worse before it gets better. He will be having the Vidaza administered as an outpatient. I'm really concerned that his levels will get so low that it will put his life in danger. He has been relatively in good shape given his condition, but if it gets worse for him, will they be giving him transfusions more often? Should he not be kept at the hospital so they can monitor him closely? Has anyone received Vidaza while having such low blood counts?

Any insight anyone may have would be greatly appreciated!

Thank you,
CatL

[bailie]

Welcome Catherine. I was diagnosed RAEB-2 then had eight cycles of Vidaza before stem cell transplant. We had to reduce the amount of Vidaza when platelets got below 20. Platelets would reach the nadir 12-14 days after each Vidaza cycle. My platelets would normally drop from about 200 (before Vidaza cycle) down to about 40 at the nadir. We wish you the best. Be sure to ask questions.

[Care.]

Hello CatL,

I think you have some very valid concerns. I cannot speak to the accuracy of your Dad's diagnosis, but getting Vidaza when blood counts are already this low might cause concern if your Dad has a heart condition or other conditions that would become more complicated by additional reduction in HGB or Platelets. For my Dad, who also was RAEB-2, the first cycles of Vidaza had more of an effect on white cells, and the effects to the red cells and platelets were temporary, for 2-3 days, before they came back up. And at first, when they did come back up, they came up rapidly.

I think the key is to stay strong enough to complete all of the Vidaza treatments each cycle, and continue with Vidaza. I think Canada has some of the most interesting studies, especially for as sick as your Dad is, that show sticking with Vedas for as long as he can tolerate it, without taking a break, is the Canadian best practice--and I wish we would have known that with my Dad.

Learning what, if any, chromosomal changes were found in your Dad's BMB will give you insight as to how well your Dad might respond to the Vidaza.

RAEB-2 is pretty serious, and I'm sure you're nervous, upset and anxious, but hang in there! My Dad had chromosomal changes that all worked against him, but he still responded very well after the first 4 cycles of Vidaza. And in his case, I think the outcome would have been better if he would have continued with more cycles before going off Vidaza the first time.

All the best to you and your family.

Care.

[Mo Sh]

Cath,

Don't be worry about the low analysis results after cycle. It's normal .. and that's how it works.
He may need some transfusions in the week after the cycle end, but it will rise again ... That's what happend with my mother who have Raeb 2 .. with 16%.

Wish you & him the best of luck.

[Catl]

Thanks to all of you for your replies. It's so comforting to be able to hear from/share with others that have had to deal with the same disease. I spoke with the coordinating nurse and his doctor today and both said not too worry about the platelets. They will be keeping a close eye and be giving him transfusions as needed. He has no bleeding even when he is as low as 6 so while they don't like him to get that low, the doctor is less concerned as he is asymptomatic (he gets really tired but that is more likely from low HGb). I pray to God that he will respond to the treatment. The next 3-4 months are going to be so stressful .

[Val702]

Hi Catl,

My 64 year old father was also just diagnosed with RAEB II. We found out on February 1. His is therapy related, he had been in remission from AML for over 5 years. We were told his MDS was a result of the strong chemo he received after being diagnosed with AML in 2011. We were told my Dad is not a candidate for a transplant, so he started vidaza this past Friday. I was also worried about him receiving the vidaza with such low blood counts.

Did you get any of the chromosomal information from the doctor? I have been going over my Dad's reports with a fine toothed comb, trying to understand and figure out what to expect. My Dad has an NPM1 mutation, which is supposedly rare in MDS and has a high chance of progressing to AML. His original diagnosis in 2011 was AML Inv. 16, which the BMB was negative for this time. I did find in my research that the NPM1 mutation usually responds favorably to vidaza.

How is your Dad tolerating the treatment so far? My Dad has been very sleepy, and his appetite has decreased. He has also been having some pretty bad headaches.

[Catl]

Hello Val,
Thanks for your message. Sorry to hear about your dad. It must be really tough to have to go through this a second time . You mention that your dad was in remission for 5 yrs. Was that from an induction and consolidation therapy? Why is he not a candidate for a transplant?

It's interesting that you ask about the cytogenetics report. I've been really frustrated because his doctor has basically refused to give us a copy of the report. I asked for it the last time we saw him the day my dad started his Vidaza and he told me I would have to make an request to archives to obtain a copy. But when I went to Archives they told me that it would take 2 months!! He did tell us when my dad was first diagnosed that he had complex abnormalities that put him in a high risk category . I really hope the Vidaza works. We have an appointment tomorrow with the bone marrow transplant team. He just finished his first round of Vidaza today and while he is very tired, he has not had any other symptoms. He has lost some weight but his appetite is still good. I think it's because he is losing muscle mass from not being active and also having made significant changes in his diet (eliminated all processed foods, added sugar dairy, eating a little meat but lots of veggies/fruits).

Good luck with your dad. Please continue to give some news.

Cat

[Catl]

Hello Val702, just wondering how things are going with your Dad's treatment? My Dad starts his second cycle on Monday. He tolerated the first cycle well with little symptoms other than fatigue and pain at the injection sites. Not sure if it means that the treatment is working, but this week was the first time since December that his platelets actually increased slightly. He was at 9,000 on Friday, so they gave him a transfusion, was at 22,000 on Monday and 27,000 today. He has been requiring platelet transfusions every 3 days or so since January, so we were excited to find out he didn't need any this week so far. His HGb is still low though (at 79) so he will receive a transfusion tomorrow. I did buy him some papaya leaves tea last week which apparently helps platelet counts, so maybe it's the tea. I guess we'll find out soon enough next week when he has another blood test. Reducing the number of transfusions is apparently better for patients that will be having a transplant as there is a risk of developing antibodies with every transplant that could impact transplant/cause GVHD.

[Sue&Dave]

Hi Catherine - my husband is mid-way through round 2 of his Vidaza treatment with no real side effects either. As a matter of fact he is feeling/looking/acting better than he has in months. Dave has the trifecta; low white, red and platelets (and a couple of crummy mutations thrown in to keep us on our toes!) His white was even as low a .7 and ANC at .3 at different times, so severely neutropenic. 10 days ago his platelets were at 17 and due to a history of bleeding he had a transfusion. Mid-way through his second round of Vidaza his platelets have been increasing and have recently jumped to 203! Crazy right? Wish I could say the same about his white and HGB. Theyare going up, but low enough where he is still neutropenic and could also require another red cell transfusion. I too put Dave on the papaya leave tea, not sure if that had any effect, but we will take good numbers as long as we can get them. Good luck to your Dad next week, I am hopeful he will continue respond to treatment!

[Catl]

Thanks Sue! Wow! That's great for your husband. I read a study from the Netherlands that showed that patients whose platelets go up early in Vidaza tend to respond better. I doubt that the tea could give him such a large improvement. My dad also is low in all three lineages, rbc, wbc and platelets. He has required blood every 7-10 days since November and platelets every 2-3 days. Is your husband planning for a SCT?

[Sue&Dave]

Catherine, ironically he was scheduled to start treatment in preparation for a SCT just about a year ago when he suffered a subdural hematoma and required 3 separate brain surgeries. He was in and out of the hospital for a 3 month period including a 3 week stay in ICU, which really opened our eyes to the rigors of a transplant! That said, it led us to our current COE and we meet with the transplant team in 2 weeks to discuss options. Unfortunately he does not have a 10/10 match, so it is either cord blood or haplo (both of our sons are half matches). We were hopeful that with the year delay a match would appear on the registry, but no such luck. We are not in a huge hurry for the transplant but understand the sooner the better. He says he would like to just enjoy the summer, which he didn't have a chance to do last year because of his surgeries. One more day of treatment this round and hope to see the same improvement that we did last time. Best to your Dad!

[Bob Macfarlane]

Hi,
Your Dad is at the right age to have perhaps served in the military in Vietnam. Possible?

[Catl]

Good luck with this cycle Sue! Sorry to hear that there isn't a match for a donor. I've read some success stories with cord or haploid SCT, so I hope for you that your transplant team will be recommending one of those procedures. Question for you regarding the Vidaza treatment - did your DH's wbc tank during his cycles so far? My dad's ANC was 1.9 at end of January and was apparently 0.2 today .

Bob, my dad did not serve in Vietnam. But he was a plumber and it is believed that he was exposed to a whole bunch of glues/chemicals for over 40 yrs.

[Sue&Dave]

Hi Cat - Yes, everything just plummeted. We were seeing an improvement after Cycle 1 and even through Day 5 & 6 of Cycle 2. But yesterday's numbers very likely mirror your Dad's. His ANC was .2 as well (I slept in a different room since I have a scratchy throat); and his platelets after skyrocketing to 230 (from 17!) have started going down too - 144 yesterday, but still good. No need for a transfusion at this point, but he has labs again tomorrow and hopefully will not need a red cell transfusion since his HGB was still good at 8.8. But man, his belly is red red red. Yesterday was the first day he complained about it and even took some tylenol to relieve the pain. He says this most recent cycle has just wore him down. Still hopeful things look up again like they did last time. How is your Dad holding out?

[bailie]

Catherine, Your Dad's experience illustrate the problem it is for the Veteran's Administration trying to decide if Vietnam (agent orange) was the cause for their MDS or AML. I know I was exposed to considerably more carcinogens (second hand smoke, exhaust, chemicals etc.) following my time in Vietnam. A great many in Vietnam smoked cigarettes which is considered a primary cause for MDS. Then also, MDS might just be the "luck of the draw".

[Catl]

Baillie, yes, I can see how it would be difficult to prove that it was the specific exposure to agent Orange 40 yrs ago instead of other post Vietnam exposure. The only way I guess it might be possible is if they were able, for example, to link exposure to agent Orange with a specific cytogenetic profile.

Sue, I guess that the decrease in wbc is likely due to the treatment then. I was concerned that it might mean disease progression. My dad had his second round of shots today and he's fine so far, just tired. His platelets didn't rise as much as your DH's but they continued to rise to 39,000 so no platelet transfusion in over a week . He also seems to get quite irritated at the injection sites. Not sure if it actually does, but I read somewhere that evening primrose oil can help. I ordered some and am waiting to receive it. Hang in there, second cycle is almost finished and hopefully his numbers will start rising again!