Too little, too late?

[JordanN]

Hi, Everyone!

I haven't posted in a long time, but I have been reading everyone's posts and wishing you all the best in your daily struggles and amazing achievements with these bone marrow failure diseases.

I have read so many threads here, but I have never seen anyone in quite the same situation my dad is in, and I wondered if anyone has had a similar experience or can at least offer some insights as to what might be going on with his MDS.

He was diagnosed with MDS RAEB 2 18 months ago and immediately started Vidaza. He has never had a positive response to the treatment (no improvement in blood values and no change in marrow blasts % (always at 18%) or cytogenics. He also had many toxicity problems with the Vidaza. So, he has been on 4 days only of Vidaza every 5 to 6 weeks for the last year since it was thought the Vidaza might just be keeping the MDS at bay.

Last month for the first time, my dad's Hgb and platelets actually rose slightly on their own without a transfusion, and he held the values for 3 weeks (very long for him). We were shocked but happily thought that, maybe, somehow, the Vidaza was finally working. Unfortunately, at the exact same time, his white count (which has never been above 1) starting rapidly rising as did his peripheral blood blast count. His WBC is now above the normal range and his blood blasts are around 40%. He also consistently has nucleated red blood cells in the peripheral blood at about 11%.

His hematologist believes that the Vidaza may be positively affecting the platelets and red cells but is having no effect on the white cells and that my dad has transformed to aml. Due to his age (81) and poor tolerance of chemo, he feels there are no options for him so he does not want to do another biopsy. He will just let the blood values dictate what we do.

So, are we getting a good response just as we are turning to aml? Or are there other explanations for the contradictory values? If anyone has any ideas or experience, I would love to hear about them. It helps to deal with the roller coaster of emotions! Thank you!

[alyasa]

I am not sure if this will help. I can only recommend to you the posts by our beloved Birgitta who was on this list and was a medical professional. The excellent links and posts helped us through many a difficulty. And I hope your dad gets back health and quality of life.

There is much uncertainty about how Vidaza works. To manage side effects, a great deal of water (like 1.5 to 2 litres per day) seems to help my dad. Others on the forum also recommended lots of water prior to, and after the vidaza cycle.

On the one hand, theory is that vidaza "aerates" the silenced genes in stem cells in the marrow. This allows for the production of cells that are functional. Hence stimulants like folic acid and B12 are not recommended (by some doctors) during vidaza treatment as it counteracts the effects of Vidaza.

The above view is not settled as Birgitta pointed us to an NIH study that showed that Neupogen administration does not cause an increase in blasts.

On other hand, the view is that cancerous cells (or stem cells) are cells not fully adapted to their environment. And because of this are "weaker" cells. Vidaza, as a cytotoxin, with a peculiar effect on blood production, then kills off the weaker cells, allowing the stronger more normal cells to produce blood.

I know this is not much help, but it has taken me time to find the contesting views. The first is the mainstream medical view. The second view is held by polymath Nicholas Nassim Taleb and is a general proposition about cancer, cancer research findings.

As far as I am aware, Birgitta did not take up vidaza treatment. But was a font of wisdom! May she have peace.

[JordanN]

Hi, Alyasa!

Thank you so much for your reply! I really appreciate your thoughts and how much research you put into your comment! We, too, have heard a number of different theories on how exactly Vidaza works, and it makes it difficult to know what is happening during treatment. I think the main thing we have learned is that MDS is not a single "problem" but a group of contributing problems which are present in varying amounts in different people so for some Vidaza fixes their primary problem whereas in others it doesn't.

I can't tell you how much I miss Birgitta! She was just a wonderful source of support and knowledge for everyone and such an elegant, compassionate, and intelligent lady!

Unfortunately, for my dad, Vidaza has been discontinued. His blast count and WBC continue to rise and his Hgb and platelets are now falling. If the Vidaza had a partial effect, it simply was not enough to prevent his transformation to AML. We are just hoping he will be comfortable and able to avoid lots of complications in the next few weeks.

I hope your father is doing well on Vidaza and able to enjoy a full quality of life, and I wish you both all the best in the future!

[Annettec]

How is your Dad? It's such a horrible disease and so very difficult to understand. My mother is on the same path so any words of wisdom or suggestions would be appreciated.

What a roller coaster of a ride! Every three months we're getting what seems to be inconsistent trends. Vidaza isn't working anymore so we're just doing 2 more, then, your mother's condition has stabilized again so we're continuing treatments, and so on and so forth. She's having her 20th weeklong treatment ( 7 consecutive days) this week. She only started transfusions about 3 months ago and for now, it seems like it's every 2-3 weeks. One or two units.

Can someone please help me to understand what this means in terms of progression.....

Ps. She chooses to live in the moment ( courageous, I envy her strength) so she doesn't want to ask her oncologist any questions.

[JordanN]

Hi, Annette!

I completely understand how you feel! As many have said on the forum, you get used to a new kind of normal where you can sort of anticipate what your loved one will need. My dad's blood values followed a fairly predictable pattern for a long time, and though his numbers were never good, we knew what to expect most of the time. We knew what Vidaza did to his Hgb and platelets, we knew when he needed a transfusion, we knew when he was getting an infection. I was always looking at his lab values terrified of the time when I knew things would eventually change but for so long he was quite stable.

As I mentioned before, my dad's lab values lost their consistency at the end of August. At first, we were hopeful because his numbers actually rose on their own, something they had never done before. We were hopeful that maybe Vidaza was starting to work though we knew that with his very reduced dose and inconsistent schedule that it was very unlikely. The scariest thing was the peripheral blood count and the WBC. Once they started to increase, it became obvious that he was converting AML. It was shocking how quickly those numbers can change and how high they can get!
I'm hoping that if your mom is still getting some stable times that her disease is not getting worse! Once my dad's disease changed it was very clear very fast that it was advancing to AML at a relentless pace.

My dad actually looked and felt the same until mid October. At that point, his WBC was around 25 and the peripheral blast count was around 65.
He just started getting one infection after another and within a couple of weeks he was on all sorts of antibiotics but nothing was helping him. His blast count was now in the 90's and WBC was around 50 even with hydroxurea. Like your mom, he didn't really want to know his prognosis, and he didn't want hospice because he was very private and had had bad experiences with them when my mom passed away. He was tired of being so sick and, as an engineer, he could easily extrapolate his lab values and figure out his own prognosis. He finally agreed to let us help him at home while "running interference" with hospice.

We lost him the day after Thanksgiving, but we were so grateful to have had him as our dad. He was amazing! He was always positive and kept his sense of humor through all of it! Sometime soon, when the tears don't run quite so easily, I will write a detailed description of his course because when we first heard his diagnosis, we were so lost and knowing one example of what someone experienced from beginning to end is so helpful.

So, we didn't get a happy ending, but my dad far outlived his original prognosis and was still able to enjoy many things until the very end. I truly hope your mom is just having a rough patch and that she still has lots of good times ahead especially with a great daughter watching out for her! Just know, that you are not alone and I do truly understand how hard it is for you! One of the worst parts of this disease is the not really knowing what is truly going on at any particular time - it is hard for everyone to live that way! Sending you hugs and hope!

[Annettec]

Good morning Jordon,
I just read your post and needed to take a moment to shed a few tears before responding. I am so, so very sorry for your loss. It's obvious you were incredibly close to your dad and that he was a wonderful father. From your words, I sense he handled his condition very much like my dear mother. She too has a great sense of humour and although it's not always easy to fall back on that, she does still manage to surprise me....
I live three hours away from her so I check in on her often by telephone and email ( like 3-5 times per day...) and i visit once per month. She's currently on her weekly Vidaza treatment, her 19th consecutive month....and i know that the treatments are taking their toll on her. Just last night, I had to laugh though....she told me that she told the oncologist yesterday " let me be clear, I don't mind being dead, it's the process to get there that I'm not so keen on"....😉
My mother has deep faith, something I truly envy as it has been such a comfort to her during this process....I hope I have half her courage when I get faced with the end of my life. As you can see, like you, I deeply love my mother and I'm not quite ready to loose her.
I hope you will stay on the forum for a while as you're so right, your wisdom and knowledge can certainly help those of us who are following the same ( horrible) path.
You now have a very special angel looking over you.... Please take comfort in knowing that your father is now at peace.....Somethign that is definitely taken away from them during this damn disease.
Take care & my deepest condolences to you and your family,
Hugs from Canada
Annette

[traceyn11]

Hi Jordon,
I just wanted to say how very sorry I am for your loss.

[Neil Cuadra]

Jordan,

I'd like to add my condolences too. It must be so hard to lose your dad like this.

Your dad does sound amazing, and down-to-earth. Engineers are very practical, aren't they? If his sense of humor helped everyone else, I'll be that having family around was what kept his own spirits up.

[Cheryl C]

Deepest sympathy Jordan. God bless you with comfort and courage.

[JordanN]

Thank you, Everyone for you kind thoughts and warm wishes! It is really helpful to hear from people who know exactly what you are going through. I wish you all the best in your continued struggles with this crazy disease!

Annette, You are so sweet! Thank you so much for all your kind words! I'll be keeping you and your mom in all my thoughts and prayers. My dad would have been so tickled with your mom's sense of humor - he said just about the same thing about "being dead" vs "getting there". Keep us posted on her progress and I hope you have a wonderful holiday with her!

Tracey, I hope things get easier for you! You've been through so much with your lungs and tummy, and I truly hope the new year brings nothing but positive changes for you and success with all of your treatments!

Cheryl, Thank you for always being there for everyone and always having great words of advice. I wish you good health for the holidays and new year!

Neil, Thank you for taking care of everyone on the forum and for managing this site! You and your wife offer hope for all of us, even those of us who didn't have the best outcome. May you and your family have continued health and a happy holiday and wonderful new year!

[Keri Anne]

My husband a Vietnam Vet was diagnosed with MDS on October 26, 2011. The doctor told us his only chance of survival was a blood transplant.
Fortunate for us one of his five siblings was a perfect match.

In March of 2012 we were sent to San Antonio Audey Murphy VA Hospital for five months. We were given the best treatment and bedside manner a patient and caregiver could ever ask for.

We were discharged on August 10, 2012 with a report of 100% remission. Tom had gone from a type O to Type A craving gummy bears. We were so excited and the doctors were so amazed by his progress. We gave all credit and glory to God.

A few weeks after returning home Tom became ill. After many trips to the hospital and several weeks stay they discovered his gallbladder was causing the problem. With his counts on the climb but not at a safe level to operate they put him on several rounds of strong antibiotics and blood/platelet transfusions. He declined daily. Eventually passed away October 23, 2012.

Shortly after his passing I was so compelled to order all of his medical documents. Something just did not add up. Early 2013 I received over 4000 pages of medical documentation. The researcher I am started at page one. It was a painful journey and process. As I got closer to the day they put him on hospice I noticed a journal entry by one of the physicians stating that he was given a Type O transfusion instead of the Type A and had contributed to his condition/relapse due to HLA mismatch...

To each of you that has lost someone to this tragic non-presumptive disease my heart goes out to each of you. I did not expect to raise our seven children alone nor enjoy our grandbabies without their pappy. I put one foot forward each day, find value in our journey and pray for a cure for the heartless bureaucracy that each of us face concerning the VA. My husband may have been 60 years young; He played golf four times a week in the Texas heat. Sundays he enjoyed taking our 12 year old to the range to hit balls. He never missed an athletic event for one of our children. His faith was incomparable. Since his passing he was not able to attend our oldest daughters wedding. He missed the birth of our two youngest granddaughters. Most all there is a whole in my heart that God is filling but its not the same. All in all there was mighty value in Tom's passing. 15 people accepted Christ at his funeral. I know he is rejoicing with the Angels.

[rar]

I have had mixed level of competence from the VA. The found and stented a 95% blockage in my LAD. 5 other civilian doctors missed it completely. When I found that I had MDS I went to the VA for their opinion. There oncologist agreed with the MDS and need for a transplant, but told me that the waiting list for transplants was so long that I would be dead before my number came up. Reminded me of my Army days. Medicare bought me a transplant a year and a half ago. So far it seems to be a cure.

Ray

[Keri Anne]

Ray I am so thankful you are doing well.
Stay strong brother...
Thank you for sharing!
Keri

[bailie]

And Keri, I went from Type A+ to Type O. I am really sorry for the loss of your husband. I too was in infantry (U.S. Army) in Vietnam.

[Keri Anne]

That is amazing...
So happy for you. Keep up the fight.
If you haven't heard check out this article...

https://www.federalregister.gov/arti...c-systems#p-82

[bailie]

Keri, thank you. I am curious to see what kind of response I get from the VA. I had MDS, then stem cell transplant and then relapse (Day +210) to AML. I am doing well considering the situation. I am sending forms to VA just to see what they say. Not holding my breath on this one, it will be interesting.

[Keri Anne]

I did hear from my attorney yesterday and he is looking into this. I'm sure it will be after the first of the year before we know anything.
Merry Christmas and Happy Birthday Jesus!